Buzz words 08/09/2010

Meissner corpuscles/krause end bulb: in papillary layer of dermis

 Langer lines, pacinian corpuscles, ruffini corpuscles: in reticular
layer of dermis
 Ceruminous (wax glands), glands of moll, mammary glands: types
of apocrine glands
 Acne ass. Glands: sebaceous glands
 Coral red fluorescence of scales under wood light: Erythrasma
 Corynebacterium (esp minutissimum): erythrasma
 Ecthyma: severe form of impetigo
 No regional lyphadepathy, little or no redness, often on face:
bullous impetigo
 Complication is acute glomerulonephritis: impetigo
 Impetigo of brockhart or barbers itch: s.aureus folliculitis
 Sty: s aureus folliculitis
 Hot tub folliculitis org: pseudomonas
 Lymphatic streaking: Superificial bact inf that extends into
lymphatics and primarly in adults: erysipelas
 How to differentiate erysipelas from cellulitis: cellulitis margins are
indistinct while erysipelas are sharply demarcated
 Verruca vulgaris: common warts: rough surface
 Type of wart with smooth surface: flat wart or verucca plana
 Wart on eye lid: filiform
 Condylomata acuminata: genital or venereal warts
 Poxvirus causes this: molluscum contagiosum
 Bimodal age distribution: molluscum contagiousum
 Henderson-patterson bodies: molluscum contagiosum
 Lesion with a central pit: molluscum contagiosum
 Lateral borders of feet: moccasin type tinea pedis
 Does not involve the scrotum: : tinea cruris
 Zoophilic or anthropophilic: tinea corporis
 Most common mycotic inf in children: tinea capitis
 Endotrix: black dot type of tinea capitus
 Mousy odor: favus type of tinea capitis
 MCC of diaper rash: cutaneous candidiasis
 Enlarged in all three dimension: nodule
 Evanescent, edematous and flat elevation: wheal (hives)
 Tree bark: lichenification
 Pus involving subQ: abscess
 Shallow abrasion with no dermal involvment: excoriation
 Cicatrix: scar
 Eschar: type of ulcer
 Over production of keratin: ichthyoses
 Mutation in ATA2A2 on chromosome 12: darrier dz (keratosis
follicularis)
 Mic app: papule shows cleft above basal layer (suprabasalar
cleft) cone of compact keratotic material: darrier dz (keratosis
follicularis)
 Karatosis follicularis aka: darrier dz
 Pink papules with silvery scales: psoriasis
 Oncycholysis and nail pitting: psoriasis
 Mic app: acanthosis with elongated rete ridges, paraketatosis and
absence of granular cell layer, mitosis above basal layer, collection
of neutrophils (munro abscesses within superficial ep: psoriasis
 Munro abscesses: psoriasis
 Koebner phenomenon: psoriasis
 Suprabasalar blisters with intercellular deposits of IgG and
complement: pemphigus vulgaris (PV)
 May be ass with MG or Lupus: pemphigus vulgaris
 Flexors and supepithelial bullae: bullous pemphigoid
 Linear BMZ of IgG and complement: bullous pemphigoid
 Ass with gluten sensitivity: dermatitis herpetiformis
 Mic app: neutrophils, eosinophils, and fibrin at tips of dermal
papillae: dermatitis herpetiformis
 Extensors and oral no oral mucosa: dermatitis herpetiformis
 Flexors and yes oral mucosae: bullous pemphigoid
 Granular deposits of IgA at tips of dermal papillae: dermatitis
herpetiformis
 Iris lesions: erythema multiforme
 Stevens Johnson syndrome: erythema multiforme
 Malar area rash: chronic cutaneous (discoid) erythematousus
 Butterfly rash: acute SLE
 Wickham stiae: lichen planus:
 Violaceous papules with white dots or lines: Wickham straie in
lichen planus
 Saw tooth pattern of deep surface of ep: lichen planus
 Colloid or civatte bodies: lichen planus
 Liquefaction degeneration at DE junction: lichen planus
 Irritating rash characterized by wheal formation: urticaria
 Mic app: scanty perivascular infilitrtates of mononuclear cells, no
increase in mast cells: urticaria
 Allergic rxn to eggs: angioedema. Aka giant urticaria *under
urticaria)
 AD disorder due to mutation in C1-esterase inhibitor: hereditary
angioneurotic edema *under urticaria)
 Affects lower extremities at sites of pressure: cutaneous necrotizing
vasculitis
 Spongiosis: allergic contact dermatitis
 Silicon breast implant rxn: granulomatous dermatitis
 Disorder of dermal CT: scleroderma
 Sweat glands and hair follicles are obliterated: scleroderma
 F:M 4:1 with non pitting edema of hand or fingers: scleroderma
 Mask like expressionless with late stage of thick dense fibrotic and
fixed skin to underlying tissue: scleroderma
 No vasculitis and no scaring: erythema nodosum
 Most common form of panniculitis: erythema nodusum
 Mic app: CT septa widening with edema, fibrin deposition and
neutrophilic infiltration, later replaced by lymphohistiocytic
infiltration with giant cells and eosinophils with (no vasculitis):
ERYTHEMA NODOSUM
 Disorder of panniculus with vasculitis and scars: erythema
induratum
 Mic app: necrotizing vasculitis affecting small to medium sized
arteries and veins in deep dermis and subcutis followed by
granulomatous inflammation and necrosis: erythema induratum
 Degenerative dz of articular cartilage: OA
 Oligoarticular: affects few joints: OA
 Eburnated: oa
 Osteophytes: joint mice: OA
 Heberden nodes: OA
 Osteophytes in DIP: Heberden nodes: in OA
 Pain and guarding of joint: synovial inflammation in OA pain
 Pain and bracing of joint: capsular inflammation (capsultitis) in OA
pain
 Radiculopathy: osteophytes (bony spurs) in OA pain
 Chronic inactivity can lead to muscle weakness and disability: OA
pain
 Muscle spasm  secondary pain: IN OA
 Non suppurative inflammatory and proliferative synovitis: RA
 Autoimmune chronic INFLAMMATORY CT dz: RA
 Immune complex formation: RA
 Prostaglandins, leukotrienes, lysosomal enz, and ROS: products of
neutrophils in RA
 Pannus: RA
 Hyperplastic synovium rich in inflammatory cells: pannus
 Rice bodies: RA: which are organized fibrin
 Mass of synovium and synovial stroma consisting of inflammatory
cells, granulation tissue, and fibroblasts: pannus in RA
 Systemic amyloidosis: extra-articular involvement in RA
 Vaculitis and inflammatory lesions in pericardium, myocardium,
lungs, pleura, peripheral nerves, and eyes: extra-articular
involvement in RA
 subQ Rh nodules: extra-articular involvement in RA
 hypergammaglobulinemia: RA
 Rf is anti what: IgG
 Ass with diuretics/aspirin/ and nicotinic acid: secondary gout* less
likely)
 Chronic hemolysis, polycythemia, leukemia, and lymphoma:
secondary gout (most commonly)
 Tophus: chronic gout
 Most imp complication in gout: renal dz : acute uric acid
nephropathy, nephrolithiasis, or chronic urate nephropathy
 Big toe: acute gout
 Chondrocalcinosis: pseudogout
 Calcium pyrophosphate crystals: pseudogout (chondrocalcinosis)
 Hydroxyapatite arthropathy: pseudogout (chondrocalcinosis_
 Legg calve Perthes dz: avascular necrosis of femoral head in kids
 Kohler dz: avascular necrosis of navicular bone
 Gaucher dz: avascular necrosis
 Steroid administration: avascular necrosis
Drugs:for a while
 Metabolism is saturable: aspirin
 Irreversible inhibitor of platelets: aspirin
 Tx for salicylism: activated charcoal , correct A/B abnormalities/
alkalize urine
 Can worsen gout: aspirin
 Reys syndrome: aspirin
 Dental pain/ oa, or RA: diflunisal (Dolobid)
 Tx for Tylenol overdose: gastric lavage with addition of sulfahydryl
cmps to replenish glutathione: N-acetylcystein (mucosil)
 Facilitiates closure of PDA: indomethacin (Indocin)
 Alternative for PDA closure if indomethacin not available: ibuprofen
(advil, motrin)
 FAP and inhibits dev of colon, breast and prostate cancer: Sulindac
(Clinoril)
 Gi effects, thrombocytopenia, agranulocytosis, nephrotic syndrome:
Sulindac (Clinoril
 This NSAID can close PDA not recommended in preggos: ibuprofen
(motrin/advil)
 Increased CV risk: naproxen (aleve/naprosyn)
 May antagonize bradykinin: ketoprofen (orudis)
 NSAID that also inhibits TNF a and NO SYNTHESIS: flurbiprofen
 Dental bone implant preservation: flurbiprofen (ansaid, Ocufen)
 Cogwheel ridgitidy, ataxia, tremor, myoclonus: flurbiprofen (ansaid,
Ocufen)
 RA + juvenile form. Adverse is nervousness and anxiety: tolmetin
(Tolectin)
 Used for post opt pain in place of morphine (opoids): ketorolac
(Toradol)
 Long half life and only single dose NSAID: piroxicam (Feldene)
 Cox2 selective combo with misoprostol: diclonfenac (Cataflam,
voltarin)
 Cox2 sel for long term tx for RA, OA or ankylosing spondylitis:
diclonfenac (Cataflam, voltarin)
 Inhibits thymidylate synthetase, and aminoimidazolecarboxamide
ribonucleotide transformylase: methotrexate (Rheumatrex)
 Crosslink’s DNA/ metabolized to phsophoramid mustard/ a DMARD:
cyclophosphamide (cytoxam, neosar)
 Decreases IL1, IL2: cyclosporine (Sandimmune, neoral)
 Avoid drinking grapefruit juice: cyclosporine (Sandimmune: neoral)
 More frequently used for transplantation than RA: a type of
DMARD: azathioprine (Imuran)
 The monoclonal antibody RA (DMARD) tx: rituximab (Rituxan)
 The monoclona antibody RA (TNFa inhibitor): adalimumab (Humira)
 Potent tnf alpha inhibitor that inhibits macrophages: infliximab
(Remicade)
 Can worsen MS: infliximab (Remicade)
 Tnf a inhibitor that can tx ulcerative colitis and juvenile arthritis and
is an recombinant fusion protein: entanercept (Enbrel)
 RA tx that prevents secondary costimulatory signal for t cell:
abatacept (Orencia)
 Reduces IgA and IgM and suppresses t cell activity: sulfasalzine
(Azulfidine)
 Inhibits dehydrooraotate dh and do not combo with methotrexate:
leflunomide (Arava)
 RA tx that can cause aplastic anemia after IM injection: gold
formulation

Bact inf of skin: favreau

 Honey colored crust: bullous impetigo
 Acute nephritis dev in 2-5% and streptococci induced cases acute
nephritis in 10-15%: non bullous impetigo
 Well defined vs less defined: impetigo to cellulitis
 Tx for impetigo: self limiting or mupirocin (bactoban)
 Tx for generalized impetigo: oral atb DOC: cephalexin (Keflex)
 Tx for generalized impetigo is penicillin allergy: azithromycin
 Ear and lower leg are high areas of infection: cellulitis
 Periorbital cellulitis: a medical emergency
 Most common pathogens causing cellulitis: GRp A stretococcus,
straph aureus
 How to dx cellulitis: clinical dx
 Tx for what dz: elevate arm above heart: cellulitis and erysipelas
 What to do if you suspect gas gangrene in cellulitis (clostridium
perfringes): xray to check for gas
 Lymphatic streaking: think erysipelas
 80% of cases of erysipelas caused by : streptococci
 FLD for erysipelas: penicillin
 Drug allergy to penicillin to tx erysipelas: erythromycin or
azithromycin
 This occurs in pts on long term atb tx for acne: Gram – folliculitis
 Hot tub folliculitis: pseudomonas
 Appears 8-48 hrs after exposure to dirty water or wet suits. :
pseudomonas folliculitis
 Seen in young adults/females and eruption is due to follicular inf by
malassezia furfur (lipophilic yeast): Pityrosporum folliculitis
 Dx of folliculitis is usually by: hx and PE alone
 Most common pathogen for folliculitis: s. aureus
 Tx for s aureus folliculitis: oral anti staph atb
 Tx for pseudomonas folliculitis: usually self limiting or Cipro
 Eosinophilic folliculitis tx: isotretinoin, metronidazole, UV-B
phototherapy, indomethacin, itraconazole
 Tx for Pityrosporum folliculitis: topical antifungal cream/shampoo
 Most common cause of carbuncles: s. aureus
 Thongs: carbuncles
 Can you transmit yeast by sex: yes
 Satellite lesions: think candidiasis
 Yeast inf in penis: candida balanitis
 Ddx for tinea vesicolor that is hypopigmented and no scale: vitiligo
 Fungal inf of palms and finger webs with scaling and erythema:
tinea manum
 Red plaque with a silvery scale: psoriasis
 Gender etiology of psoriasis: m=f
 Auspitz sign: pinpoint bleeding seen when cutting into edge of
psoriasis
 Tx for psoriasis: topical steroids
 100% TQ: herald patch: pityriasis rosea
 5 ps of lichen planus: pruritic, purple, papules, planar, polygonal
khin skeletal system
 cyst that contains amorphrous protein like material and lined by
fibrous tissue, few giant cells, hemosiderin-laden macrophages,
chronic inf cells and reactive bone: solitary(unicameral bone cysts)
 curettage and insertion of bone chips-curative: solitary(unicameral
bone cysts)
 most tumors appear near: metaphysis
 more than 80% of primary tumors occur in what bones: distal
femur and proximal tibia
 involves skull and facial bones such as frontal sinus (most common
site): osteoma M>f
 gardner syndrom: osteoma M>F
 xray finding of central radiolucent area (nidus): osteoid osteoma
M>F
 non painful and predominantly affects vertebrae and long bone and
m=f: osteoblastoma (giant osteoid osteoma)
 most common primary malignant tumor representing 1/5 th of all
bone malignancies: osteosarcoma (osteogenic sarcoma)
 may arise in ass with pagets, fibrous dysplasia, enchondroma, or
previous exposure to irradiation: osteosarcoma (osteogenic
sarcoma)
 tumor suppressor genes such as p53 or 2/3rd due to Rb mutation:
osteosarcoma (osteogenic sarcoma)
 conmans triangle: osteosarcoma (osteogenic sarcoma)
 sun ray appearance on xray : osteosarcoma (osteogenic sarcoma)
 rare variant of osteosarcoma that occurs more in females:
juxtacortical osteosarcoma
 non hereditary disorder with multiple enchondromas in metaphysis
and diaphysis of various bones: ollier dz (enchondromatosis)
 familial multiple form ass with hemangiomas in skin and viscera:
Maffucci syndrome
 codman tumor: aka: chondroblastoma: M in second decade of life
and affects epiphyseal areas of bones
 ass with trisomy 7: chondrosarcoma
 ass with rearrangement of chrom 17: high grade chondrosarcoma
 large bossilated mass that surrounds the base of an
osteochondroma and invasion in bone: peripheral chondrosarcoma
 slow growth may lead to compression of lumbosacral plexus and
thus paraplegia: peripheral chondrosarcoma
 gender preference for juxtacortical chondrosarcoma vs
osteosarcoma: chondro: M vs osteo: F
 snowstorm appearance: condrosarcoma
most frequent primary sites are Males: bronchus/prostate and F:
breast: metastatic bone tumors
first sign of neoplasm’s: back pain
periosteal onion skinning and sunburnt pattern of maligant
metastatic bone tumor: ewing sarcoma
tumor cells contain abundant glycogen: ewring sarcoma
osteoclastoma:aka: giant cell tumor of bone
soap bubble appearance : osteoclastoma:aka: giant cell tumor of
bone
 spindle stromal cell: osteoclastoma: aka giant cell tumor of bone
 osteitis deformans: paget dz of bone
 irregular thickening and softening of the bones: paget dz: osteitis
deformans.
 Slow virus: Paramyxovirus: paget dz: osteitis deformans.
 Woven bone with mosaic pattern: initial stage of paget dz: osteitis
deformans.
 Coxa vera and anterior bowing: paget dz: osteitis deformans.
 Deafness, height distortion, coarsening of facial bones (leontiasis
ossea) paget dz: osteitis deformans.
 Serum alkaline phosphatase elevated is the most useful lab test to
dx: paget dz: osteitis deformans.
 Ass with skin pigmentation and endocrine distrubances: fibrous
dysplasia
 Most common endocrine abnormality: Albright syndrome
 Pigmented macules (café au lait spots) usually over buttock, back
and sacrum: Albright syndrome
 Histological features of woven bone that looks like Chinese
characters: fibrous dyspasia
 Clubbing of digits: hypertrophic osteoarthropathy

Few hem questions:

 Pt has some mile petechia and bruising: this means what: low
platelet count
 TQ: hallmark of the dz requires both pancytopenia and acellular
bone marrow filled with fat, sustained (often irreversible), and
caused by stem cell injury: aplastic anemia: *the PLASTIC surgeon
put the FAT in the PAN.
 What is the genetic predispositon for aplastic anemia: HLA-DR-2 (
 exposure to benzene: Aplastic anemia
 name the dz: pancytopenia, acellular (or marked hypocellular )
bone marrow, CFU-GM and BFU-E decreased, decreased long term
culture initiating cells to less than 1%, flow cytometry rules out ALL
or hairy cell leukemia: Aplastic anemia
 how would you dx apastic anemia: get a CBC to see pancytopenia,
get a BM biopsy to see fatty infiltration
 whats the most imp tx for survival of aplastic anemia: bone marrow
transplantation
 whats the prognosis of Aplastic anemia: now 75%, but 25% severe
graft vs host (GVH), 15% relapse, 25% progress to PNH, MDS, or
AML
 what are the Ddx of aplastic anemia: (4): (AMP-H): ALL,
myelodysplastic syndrome, PNH, Hairy cell leukemia
 what dx test of choice for AA: bone marrow aspirate and biopsy
 what are you looking for on the bone marrow aspirate or biopsy:
spicules with empty fatty spaces with hypo-cellularity
 whats the best therapy for AA: remove oftending agent, blood
transfusion, BM transplant, immunosuppresents (cyclosporine, anti-
thymocye globulin), corticosteroids, bone marrow stimulants (GCSF
or other cytokinesn, epo. Tincture of time
 myelo means: all formed elements of blood
 schisto means: fragmented cells
 pancyto means: all the cells
 pioklio: change in shape
 reticulo: early rbc precursors
 blastic: early
 what does a CBC test: HHRRWWPB: hct, hgb, rbc count, rbc
indices, wbc count, wbc types (differential), platelet count, blood
smear
 MCV: average RBC size
 MCH: Hb amt per RBC
 MCHC: Hb amt relative to size of cell (hb concentration) per RBC
 ICT vs DCT: ICT= indirect chulmbs test= tells you atc is in the
plasma vs DCT: direct chulmbs test= atb on the RBC: D or D**
 4 d’s of quantitative thrombocytopenia: decreased production,
dilution, distribution, increased destruction.
 Myeloid vs lymphoid: myeloid is from the bone marrow where as
lymphoid is from the LN, thymus and spleen. Myeloid induces:
precursors for granulocytes (neutrophils, basophils, eosinophils,
monocytes, rbc, platelets. Lymphoid induces LYMPHOCYTES
 Acute vs chronic: acute young malignant cells: predominaly blasts
vs chronic in which mature malignant cells predominate
 Dz of adults, stem cell disorder, mature cells predominate, and BM
shows “hypercellularity”: myeloproliferative disorders
 TQ:Multiple myeloma, light and heavy chain dz, benign monoclonal
gammopathy, walenstroms macroglobulinemia, amyloidosis,
plasmacytoma, plasma cell leukemia, monoclonal gammopathy of
undertermined significance Plasma cell disorders.
 Pt (prothrombin time) or PTT (partial thromboplastin time:
o Extrinsic pathway:factors 2, 5, 7, 10 or fibrinogen: PT
o Factors: 8, 9, 11, 12 and rarely von willebrands factor: PTT
 TQ: protein C or protein S def or HIT antibody: hypercoagulable
states
Stopped at start of hemolytic anemia lecture
 Pentad of TTP: microangiopathic hemolytic anemia,
thrombocytopenia, renal failure, mental status changes, fever
 Tx for TTP: plasma exchange
Panavellil heme dugs
 Tx for iron toxicity: deferoxamine”
 Tx for ACD: EPO
 Tx for CRF: chronic renal failure: EPO
 Tx for bone marrow deficiency states: CSF (colony stimulating
factor)
 Production of RBC by what molecules: EPO
 Where is epo produced: kidney
 What is epo’s trigger for release: low o2 content which then
stimulates__________ (stem cells to differentiate to
proerythrocytes) , increases in mitosis, increase release of
reticulocytes, induces hb formation

 Where is the majority of iron absorbed: in the duodenum in the

ferrous state (fe2+)
 Transport plasma protein that delivers iron the bone marrow for the
incorporation into RBC hb: transferrin
 Where is iron stored: in macrophages in the reticuloendothelial
system as ferritin or hemosiderin
 What do RBC break down into: aa and porphyrin
 Bilirubin is released into the plasma where it binds to albumin and
is transported to the LIVER for what rxn: glucoronide conjugation
and excretion via bile
 Tx for IDA: iron deficiency anemia: ferrous sulfate, ferrous
fumarate, ferrous choline citrate, ferrous gluconate, polysaccharide
iron complex
 How does orange juice affect IDA tx:2x the absorption
 How does tea or coffee affect IDA tx: ½ the absorption
 Therapeutic doses of iron increase hb by : 1g/wk
 Parental iron forms: iron dextran, INFeD, dexferrum, pri-dextra,
sodium ferric gluconate complex-ferrlecit, iron sucrose (Venofer)
 in IDA pts with MALABSORPTION SYNDROMES or pts INTOLERANT
TO ORAL IRON PREps a combo of what is given: ferric hydroxide
and dextran (iron dextran) containing 50mg/ml iron im or iv by
multiple slow injections
 what do the reticuloendothelial cells do to the iron dextran:
phagocytize it
 what is an adverse affect of IM injection of iron: tissue necrosis or
atrophy
 what are pre existing immune conditions that my predispose pts for
high risk adverse rxns for parenteral iron forms: SLE and RA
 the newer form of parenteral iron for chronic renal disease: Ferrlecit
(sodium ferric gluconate complex)
 exchange mechanism seen in which drug: iron sucrose (Venofer)
 what is the form indicated for IDA in chronic hemodialysis pts who
are receiving EPO therapy: iron sucrose (Venofer)
 whats an advantage of parenteral vs oral: faster
 GI upsets, discoloration of feces (black): adverse rxns to parenteral
iron
 What are the mech of toxicity for iron toxicity: 1 direct corrosive
effec on mucosal tissue resulting in necrosis and perforation, 2.
Cellular toxicities due to lactic acidosis and necrosis
 Clinical presentation is bloody diarrhea, massive fluid loss and blood
loss into gi tract causing shock, renal failure and death: iron toxicity
 What is metabolic acidosis ass with: iron toxicity even after survival
 What is the chelating agent used for: its deferoxamine and its used
for iron toxicity
 Orange or pink red urine: tx for iron toxicity: deferoxamine
 Cyanocobalamin: b12
 Cobalamin is absorbed by both: intrinsic factor dep and
independent routes
 Malfunction of the parietal cell: decreases intrinsic factor which
decreases cobalamin absorption (b12) which causes pernicious
anemia
 Pernicious anemia: b12
 Acquired vs congential pernicious anemia: acquired if decreased
production of intrinsic factor. Congenital if dysfunction of intrinsic
factor
 B12 transport protein: transcobalamin II
 Addisonian anemia aka: pernicious anemia
 Tx of pernicious anemia (addisonian anemia): oral b12 therapy
 Which anemia can be tx with intranasal formulations: vit b12 def
with intranasal cyanocobalamin formulations.
 Whats hydroxocobalamin: preferred for b12 anemia due to highly
protein bound and remains longer in circulation
 Hyperuricemia, hypokalemia, and sodium retention and rebound
trambocytosis leading to thrombotic events seen in: b12 induced
reticulocytosis
 Schilling test: test to determine b12 def
 Whats ass with colon cancer: excess homocysteine seen in folic acid
def anemia
 Two drugs that can block dihydrofolate reductase and lead to folic
acid def anemia: methotrexate and trimethoprim
 Are folates toxic: no known toxitcity
 What is the major difference seen in folate and b12 def: in folate
def there is an absence of neurological manifestations
 Tx for megaloblastic anemia: folate and cyanocobalamin
 What is the glycosylated protein: EPO
 Tx of renal failure anemia, aids anemia, ACD such as metastatic
cancer and RA: epo
 How is epo administered: IV or SUBQ
 Epo effect of rbc: increases levels
 Epo effect on hct: increases and activates syn of hb
 What is epo not indicated for: pts needing immediate correction of
severe anemia
 What is the major side effect of epoetin therapy: elevated diastolic
pressure (Hypertension)
 What causes seizures, thrombosis or iron deficieny: EPO
 What does Neupogen (for wbc def) do: increases total neutrophil
count
 Tx for white blood cell def: G-CSF (Neupogen), Leukine, or
neulasta)
N. ditchek rheumatoloical problems lecture
 What should lead to correct dx in 85% of rheumatologic problems:
hx and pe.
 Functia laesa: loss of function
 Rigor: stiffness
 Erythema, soft tissue swelling, sausage digits, and nail changes
seen in : psoriatic synovitis
 Podagra: gout in the big toe
 Vesicles and pustules due to gonococcal arthritis: pustular synovitis
 Pip vs dip: pip: bouchards, dip : heberdens: both in OA
 Non inflammatory: OA
 Inflammatory : RA, SLE, psoriatic arthritis
 Features of arthritis: inflam, pain in all motions (active and
passive), pain around joint, and decreased rom
 What are some routine arthritis screening tests: acute phase
reactant (ESR and C-RP), RF, and ANA
 ANA
PATTERNSxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxx
o Hemogenous histones/dna: SLE or drug effect
o Speckled ENA: MCTD, SLE, sjogrens syndrome,
poly/dermatomyositis
o Nucleolar RNA ass angtigen: scleroderma
o Peripheral (rim) DNA nuclear antigens: SLE
o Centromeric nuclear antigens Limited scleroderma (crest
syndrome)
 AUTOANTIBODIES IN RHEUMATIC DZ
 Anti-dsDNA: SLE
 Anti-histone: SLE, drug induced lupus
 Anti-ENA: (sSm or RNP (ribonucleoprotein): SLE MCTD or SLE
 Anti ss-a/ro: SLE, neonatal lupus, sjorgrens syndrome
 Anit ss-b/ro: sjorgrens, sle, neonatal lupus
 Anti centromeric: limitied scleroderma (crest syndrome)
 Anti scl-70: diffuse scleroderma
 Anti jo 1: poly/dermatomyoctis, raynauds phenomenon’s
 Anti PM scl: polymyositis/ scleroderma overlap syndrome
 Anti mi 2: dermatomyositis

 Special arthritis tests:

o Complement levels: CH 50, C3, C4
o HLA: b27, D locus alleles
o Antineutrophil cytomplasmic antibody (ANCA)
o Cryoglobulins
o Lyme titers
 Routine lab studies for rhematologic studies
 o Cbc
o Chemistries
 Glucose
 Liver function test
 Cpk
 Uric acid
 Urinalysis
 What are the causes of hemarthrosis (bleeding into joint)
o Trauma
o Bleeding disorder
o Villonodular synovitis
o Pyrophosphate arthropathy
o Charcot joint
o Resolving inf
o Contamination during aspiration
 Synovial effusions
o Clear colorless viscous with leukocytes <200, <25% pmn:
normal
o Clear, yellow, viscous with leukocytes 200-2000, <25% pmn:
non inflammatory: ie OA
o Cloudy, yellow, watery , glucose may be low with leukocytes
2000-100,000, >50% pmn: inflammatory: RA
o Purulent, glucose very low with leukocytes >80,000 and
>75% pmn: septic: bact inf/fungal inf, mycobacterial inf, or
acute crystal synovitis
 Types of crystals found in acute gout and topheacous gout
monosodium urate monohydrate
 Calcium pyrophosphate in : pseudogout, destructive arthropathy
 Basic calcium phophate: acute calcific periarthritis, acute arthritis,
destructive arthropathy (Milwaukee shoulder/knee)
 Calcium oxalate: acute, subacute arthritis
 Lipids (fat): acute arthritis
 Cholesterol: asymptomatic
 Negative birefringence: urate crystals in gout
 Positive birefringence : calcium pyrophosphate crystals in
pseudogout
Vasculitis
 Whats the key to dx for vasculitis: clinical suspicion
 Pulseless, blindless, cva, and syncope: large vessel symptoms
 CAD, Renal artery CVA, mononeuritis (writs/foot drop): medium
vessel
 Palpable purpura and raynauds/ gangrene: small
 The only medium vessel one: polyarteritis nodosa
 The two large vessel ones: takaysu (rare) and most common
temporal arteritis
 Past TQ: causes of leukocytoclastic vasculitis aka hypersensitive
vascultis or allergic vasculitis: viral/ bact/ atypical, meds, lupus,
idiopathic, insect bites
 Livedo reticularis: mottled erythema seen in : small vessel
vasculitis
 Necrotizing (caseating) granulomatous vasculitis, 1.3:1 in M;f,
>95% in caucasians in 20-40s with tx of glucocorticoids +
cyclophosphamide: wegeners granuloamatosis (sm vessel)
 What is ELK: main sites for wegeners granulomatosis: Ent/ upper
rt: optic, otic, sinusitis, Lower rt: lungs, Kindey
 Whats the lab test for wegeners granulomatosis: C-ANCA
 TQ: what is Henoch Schonlein tetrad: (Ga^2p)
o Glomerular nephritis
o Ab pain
o Arthritis
o Purpura
 Markers of inflammation: Elevated ESR and C-RP
 Hematologic changes in vasculitis: leukocytosis usually with
neutrophila, anemia, thrombocytosis
 High RF with low complements: hepatitis related PAN, RA, or
cryoglobulinemic vasculitis
 Past TQ: ANCA ass dz: wegners, microscopic polyangiitis, chrug-
strauss syndrome, drug induced anca ass vasculitis
 Weight loss >4kg, livedo reticularis, testicular pain or tendernous,
myalgias, weakeness or leg tendernous, mononeuopathy or
polyneuropathy, diastolic bp>90, elevated BUN/creatinine, hep b
virus, arteriographic abnormality, bx of small or medium artery
containing PAN: polyarteritis nodosa (the only medium vessel
vasculitis)
 Pts at least 50 and usually white, pain at least 1 month in shoulders
and pelvis, severe morning stiffness and gelling: polymyalgia
rheumatica (large vessel)
 HA, diplopia, anaurosis, jaw claudication: cranial arteritis
 Giant cell arteritis aka: temporal arteritis
 TQ:Elevated Westergren esr >59: temporal arteritis
 Tx for temporal arterits: high dose steroids
 Dx of temporal arteritis: must biopsy temporal arteries bilaterally
 Fragmentation of the intima layer of vessel: temporal artertis
 TQ: the most dangerous outcome of TA: ischemic optic neuritis:
demands immediate tx with high dose steroids
Jones skeletal MSK pathology
 Connective tissue covering of bone: periosteum
 Mononuclear cells that syn collagen and ground substance:
osteoblasts
 Osteoblasts that become embedded and incorp into bone matrix
osteocytes
 Multinucleated cells found along cortical endosteal surface and
trabeculae in scalloped bays called howships lacunae osteoclasts
 Howships lacunae: osteoclasts locations
 Haversian system: compact bone
 Basic unit is trabecula: cancellous bone
 Collegen laid down in parallel array: lamellar bone
 Collagen laids down haphazardly : woven bone
 Endochondral ossification: long bones
 Intramembranous ossification: flat bones
 Dz characterized by increased porosity of skel reduced bone
mass: osteoporosis
 Bone mass determined by : vit D receptor allele, genes for collagen
1A1, estrogen rec, Insulin like growth factor 1 and its binding
protein, physical activity, diet, hormones
 When is maximal skeletal mass attained: after 3rd or 4th decade
 When does postmenopausal osteoporosis occur: 1 decade after
menopause
 Decreased estrogen levels in post menopausal osteoporsis results
in: increased secretion of IL1, IL6 and TNF by blood monocytes and
bone marrow cells (these cytokines activate osteoclasts)
 What causes senile osteoporosis: osteoblasts reduced replicative
and biosynthetic potential
 Morphology: affects the cancellous compartment of vertebral
bodies: post menopausal osteoporosis
 Trabeculae are thinned: post menopausal osteoporoiss
 Cortex is thinned: senile osteoporois
 Haversian systems are widened: senile osteoporosis
 Cannot be detected in plain radiographs until 30-40% bone mass is
lost: osteoporosis
 Best dx of osteoporosis: dual energy absorptiometry and
quantitative computed tomography which measure BONE DENSITY
 Major function is maintenance in normal plasma levels of ca and
phorphorus: vit D
 This is required for absoption of ca from small int: vit D
 Vit d def results from: dietary deprivation, fat malabsorption, lack
of sun exposure, chronic renal failure:
 Vit d def or phosphate depletion in kids: rickets
 Vit d def or phosphate depletion in adults: osteomalacia
 Deranged bone: rickets
 Undermineralized bone: osteomalacia
 Wide osteiod seams: rickets or osteomalacia
 Rachitic rosary (swollen costochondral junctions of ribs): rickets
 Pigeon chest: rickets
 Bowing of legs and arms: rickets
 Xray will show thickend, irregular, lobulated epiphyseal plate:
rickets
 Osteopenic x ray finding: osteomalacia
 Compression fx and decreased bone thickness: osteomalacia
 Dev of secondary hyperparathyroidism: osteomalacia (due to low
serum ca which stimulates parathyroid glands)
 What causes 80% of primary hyperparathyroidism: adenoma of the
parathyroid gland
 Osteitis fibrosa cystica: refers to the anatomical changes of severe
hyperparathyroidism
 Morpholgy is increased osteoclast affects cortical bone, dissecting
osteitis (in cancellulous bone what looks like railroad tracks), xray
finding of osteopenia (decreased bone density), marrow space
replaced by fibrovascular tissue, and widening of haversian canals:
hyperparathyroidism
 Bone loss predisposes to microfx and secondary hemorrhages:
hyperparathyroidism’s
 Brown tumors: hyperparathyroidism
 Hallmark is generalized osteoitis fibrosa cystica: von
Recklinghausen dz of bone which includes increased bone
cell, peritrabecular fibrosis, and cystic brown tumors: severe
hyperparathyroidism
 CRF results in phosphate retention and hyperphosphatemia: renal
osteodystrophy
 What does the hyperphosphatemeia in renal osteodystrophy
trigger: secondary hyperparathyroidism hypocalcemia develops
intestinal ca absorption decreased due to high levels of phosphorus
inhibiting renal hydroxylase, PTH secretion increases with a
decrease degration and excretion of PTH which results in secondary
hyperparathyroidism which increases osteoblast activity. Metabolic
acidosis is ass with renal failure which stimulates bone resorption
and the release of calcium hyroxyapatite from matrix
 Aluminum: renal osteodystrophy
 What are sources of aluminum: dialysis sol. Or oral al phosphate
binders.
 What does aluminum do: interferes with deposition of calcium
hydroxyapatite and thus promotes osteomalacia
Kimmel lecture 1 RA and OA
 Genetic predisposition: OA
 Worse with use. Improves with rest: OA
 Bony changes localized to weight bearing surfaces/ joints: oa
 Osteophytes: oa
 Hylans: tx for oa
 Decrease life expectancy: steroids
 Increase life expectancy: DMARDS
 7 criteria for RA: (must be present for >6 wks
o 3 jnts
o hands and wrists
o AM stiffness
o Symmetry
o Nodules
o Rf +
o X ray changes
 Immunological disorder: RA
 Worse at rest better with use, inflammatory/ systemic complaints:
RA
 Lab findings for RA: RF, CCP, ESR, CRP
 Xray finding for RA: juxta-articular osteoporosis, jt space narrowing
diffusely and errosions
 DMARDS: increase life expectancy
o Mild:
 plaqenil (hydroxychloroquine)
 azulfidine (sulfasalazine)
o stronger
 Rheumatrex/Trexall (methotrexate)
 Avara (leflunomide)
 Biological DMARDS
o Inhibitors of TNF
 Soluble receptor: etanercept (Enbrel)
 Monoclonal ab: adalimumab (Humira)
 Golibumab (simponi)
 cerulizumab (cimzia)
 infliximab (Remicade)
o IL1 inhibitor: anakinra (kenneret)
o IL6 inhibitor: tocilizumab (actimra)
 o B cell inhibtor: rituximab (Rituxan)
o T cell inhibitor: abatacept (Orencia)
o oral biological DMARDS: protein kinase inhibitors
 abnormalilty in collagen type II gene (aberrant COL2A1 gene): OA
 no ass with HLA: OA
 HLA-DR4: RA in white people
 HLA-DR1, DR10, DRw6: RA in non white
 Life expectancy of RA: 10 years less than normal
 In OA which cause decrease in matrix syn: IGF 1, TGF beta
 In oh which cause increase in matrix degradation: cytokines, enz,
NO
 Major cell type in RA in synovial fluid: neutophils
 Major cell type in pannus: t lymphocytes and macrophages
 Minor cell types in pannus: fibroblasts, plasma cells, endothelium,
dendritic cells
 Crepitus: oa
 Polyarticular: RA
 Joints involved in primary OA: cervical/lumbar vertebrae, hip,
dip/pip, 1st CMC, knee, 1st MTP
 Diagnostic joints for early ra: wrist, 2 or 3rd MCP, MTP
 Lab result of ESR <20 : OA
 Most specific xray finding for Oa: osteophytes
 Can Rf be negative in early RA: yes
 When does symmetry of joint involvement occur in RA: late ra
 RF+ but ANA -: RA with no SLE
 Spondyloarthropathy lab finding: RF- and ANA –
 Erosions: RA
 Glucosamines use: OA ONLY>>>>>
 Fentanyl patches, codeine, oxycodone, propoxyphene, or morphine:
opioids
 Which opioid is long acting an will have fewer CNS side effects and
less addictive potential: morphine
 Cox-2 specific: celecoxib (celebrex), vioxx and Bextra
 Selective cox 2: meloxicam (Mobic), and nabumetone (Relafen)
 Hylans: hyalgen, Synvisc, orthovisc, Supartz, euflexxa
 What are the indications for hylans: tx of knee or hip Oa in pt
unable to respond to steroid injections. DO NOT USE FOR small
joints or inflam arthritis (RA)
 Hypopigmentation: steroid side effect
 Diabetes flare: steroids side effect
 What are the indications for joint replacement: persitent pain
despite medical PT, loss of joint function, fracture
 Absolute contraindications for joint replacement: infection
 Relative contraindications for joint replacement: age, obesity, poor
health, poor bone stock, deformities, prior infection
 Which ones slow dz progression in RA tx: DMARDS
 DMARD side effects TQ
o Macular damage: hydroxycholorquine
o Myelosuppresion, GI: Azulfidine
o Gold oral: myelosuppresion, rash, proteinuria, GI
o Leuflunomide: hepatotoxic, GI no in preggo
o Methotrexate: hepatoxic, pulmonary, myelosuppression, No in
preggo
 Contraindications are MS or TB inf, CHF, vaccines (zoster): tna
antagonist
 Criteria for dx of adults stills dz:
o All required:
 Fever >39 c
 Arthralgia or arthritis
 Rh factor <1:80
 ANA <1:100
o In addition to any of the following
 Wbc >15000
 Stills rash : faint salmon colored rash on trunk or UE
 Pleuritis
 Pericarditis
 Hepatomegaly or splenomegaly or generalized
lymphadenopathy
 Complication of adult stills: hepatic necrosis
 Tx for adult stills: biological tx IL1 inhibitor: anakinra (kinneret)
 Dx of feltys syndrome
 o Seropositive rheumatoid arthritis
o Splenomegaly
o Granulocytopenia
 How long should you use contraception after on MTX: 3-6 months
 How long should you use contraception after on leflunomide (Arava)
2 years
Khin msk skeletal system
 80% of primary bone tumors occur in: distal femur or proximal tibia
 frontal sinus: osteoma
 gardners syndrome: osteoma
 nidus: osteoid osteoma: usually LE and small bones of foot
 pagets, fibrous dysplasia, enchondroma or exposure to irradiation
association: osteosarcoma
 codmans triangle: subperiosteal elevation seenin osteosarcoma
 sun ray appearance on xray: osteosarcoma
 codmans tumor: aka chondroblastoma
 codmans tumor (chondromblastoma affects
_____________(epiphyseal area) where as chondromyxoid fibroma
affects ___________(metaphyses)
 ass with trisomy 7: chondrosarcoma
 ass with rearrangement of chromosome 17 high grade
chondrosarcoma
 large bossilated mass: peripheral condrosarcoma
 gender preference for juxtacortical chondrosarcoma vs juxtacortical
otsteosarcoma: chondro: men, osteo: women
 snowstorm appearance condrosarcoma
 MC sites for metastatic tumors of bone in M: bronchus and
prostate, F: breast
 Onion skinning and sunburnt pattern: ewing sarcoma (a type of
metastatic bone tumor)
 Ass with reciprocal translocation t(11;22): ewing sarcoma (a type
of metastatic bone tumor)
 Tumor cells with abundant glycogen: ewing sarcoma (a type of
metastatic bone tumor)

  Soap bubble appearance: giant cell tumor of bone (osteoclastoma)
 Gender pref for giant cell tumor of bone (osteoclastoma): females
slightly
 Mic app of giant cell tumor of bone (osteoclastoma): composed of
plump spindle cells among scattered multinuclearted giant cells.
The neoplastic cell is the spindle stromal cell
 Irregular thickening and softening of bone: paget dz (osteitis
deformans)
 Woven bone with mosaic pattern: paget dz (osteitis deformans)
 Vertebral compression, height distortion, coarsening of facial bones
(leontiasis ossea): paget dz (osteitis deformans)
 Serum alkaline phosphatase elevated and is the most use lab test
to dx this dz: paget dz (osteitis deformans)
 Ass with skin pigmentation and endocrine disturbance: fibrous
dysplasia
 Most common endocrine abnormality: Albright syndrome
 Café au lait spots over buttocks, back and sacrum: Albright
syndrome
 Lucent ground glass appearance and bones appear as Chinese
characters : albrights syndrome
 Cellular fibroblastic tissue with scattered lipid laden macrophages
and multi nucleated giant cells, no bone formation or anaplasia:
nonossifying fibroma (fibrous cortical defect
 Clubbing of digits: hypertrophic osteoarthropathy
Jones infection inflammatory and reactive arthritic conditions
 Most common organisms of infectious arthritis (PEGS3)
o Pseudomonas
o Ecoli
o Gonoccus
o Staph
o Strep
o Salmonella
 Main causative agent for infectious arthritis in children and adults:
s. aureas
 Same as above but in late adolescence and young adulthood:
gonococcus
 If sickle cell more prone to infect arthritis by this organism:
salmonella
 Sudden acutely painful, hot and swollen joint, restricted rom, fever,
leukocytosis and elevated ESR: infectious arthritis
 Disseminated gonococcal inf sites vs intravenous drug abuse sites:
gon: single joint where as IV drug is axial articulations such as
spine, sacroiliac or sternoclavicular
 Chronic progressive/ monoarticular dz occurring in all age groups:
tuberculous arthritis
 What joints are affected in tuberculous arthritis: weight bearing
joints
 Mycobacterium stain: red (fite stain)
 Clinical lab aids in dx
o Gram stain:
 Always performed as routine
 75% sen for staph
 50% sen for most gram –
 <25% for gonococcal
o blood cultures
 obtained before atb given
 positive 50% for staph aureus
 less frequently + for other org
o synovial fluid
 75-95% sen for non gonococcal joint inf
 can be turbid, serosanguineous or purulent
 high number of neutrophils
 protein/ LDH/ elevated
 glucose low
 fluid should be examined for crystals to r/o other causes
 cultures are + > 90% of time
 non inflammatory:
o oa, traumatic arthritis, PVNS
 inflammatory
o RA, sle, reiter syndrome, rheumatic fever, acute crystal
induced arthritis
 Purulent (infectious) effusion
o Bac/fungal/tb joints
 Hemorrhagic effusions
o Traumatic, PVNS, synovial hemangiomas, hematologic
disorders, thrombocytopenia, anticoagulant therapy

 Bamboo spin in : ankylosing spondyloarthritis
 What are the seronegative spondyloarthropathies (3)
o Ankylosing spondylitis
o Reactive arthritis (reiters syndrome and enteritis ass arthritis)
o Psoriatic arthritis and arthritis ass with IBD (ulcerative colitis
or crohn dz)
 What do the seronegative spondyloarthropathies have in common:
ass with HLA-b27
 Rheumatoid spondylitis and marie stumpell dz aka: ankylosing
spondyloarthritis
 Which one especially affects the sacroiliac joint: ankylosing
spondyloarthritis
 Gender preference for spondyloarhtris: men 2-3x>F
 What is the SI joint infiltrated with in ankylyosing spondyloarhtritis:
CD4 and CD8 t cells, macrophages and high tnf Alpha
 Inflammation of tendinoligamentous insertion sites and ossification
aka syndesmophytes: ankylosing spondyloarthritis
 Complications in this dz may include fx of spine, uveitis, aortitis,
and amyloidosis: ankylosing spondyloarthritis
 Defined as an episode of non infectious arthritis of the appendicular
skeleton that occurs within one months of a primary inf localized
elsewhere in the body: reactive arthritis
 Ass with genitourinary (chlamydia) and GI tract inf (shigella,
salmonella, Yersinia, campylobacter): reactive arthritis
 The triad of arthritis, non gonococcal urethritis or cervicitis, and
conjunctivitis is called: Reiter syndrome
 Define reiter syndrome: The triad of arthritis, non gonococcal
urethritis or cervicitis, and conjunctivitis
 What are most affected joints in reactive arthritis: ankles, knees
and feet often asymmetrical pattern
 Sausage like finger or toe: reactive arthritis
 Inflammatory balanitis (circinate), conjunctivitis, cardiac conduction
abnormalities, aortic regurgitation: extraarticular involvement in
reactive arthritis
 Ganglions, synovial cysts, and osteochondral loose bodies aka:
reactive tumor like lesions
 What do the above result from: trauma or denerative processes and
they are more common than neoplasm’s
 Involves the synovium of a joint, involves one or more joints
DIFFUSELY, and happens in 20-40s in m=f: pigmented villonodular
synovitis (PVNS)
 Occurs as DISCRETE nodule on tendon sheath in 20-40s and m=f:
giant cell tumor of tendon sheath( GCT)
 Giant cell tumor of tendon sheath aka localized nodular
tenosynovitis
 Grossly these lesions are red brown to mottled orange-yellow:
PVNS and GCT
 Resembles a small walnut: GCT
 Tangled mat of red brown folds and finger like projections and
nodules in the knee: PVNS
the tumor cells are POLYHEDRAL, moderately sized and resemble
synoviocytes: GCT and PVNS
 Hemosiderin depostits, FOAMY macrophaes, multinucleated giant
cells, and zones of sclerosis: GCT and PVNS
pts complains of pain, locking and recurrent swelling of knee: PVNS
 Which one is aggressive and painfull and which one is solitary,slow
growing and painless mass: aggressive +painful =pvns. Solitary
slow growing: GCT
 Frequently involves the tendon sheaths along wrists and fingers and
is the most common mesenchymal neoplasm of the hand: GCT
  Where do synovial sarcomas occur: majority dev in deep soft tissue
in vicinity of the large joints of the extremities, 60-70% occurring in
LE, esp knee and thigh
 Biphasic and monophasic: synovial sarcoma
 Hallmark is eh dual line of differentiation of the tumor cells (ie
epithelial like and spindle cells): synovial sarcoma
 Epithelial like cells are cuboidal to columnar and form glands or gro
in solid cords are aggregates: synovial sarcoma
 Spindle cells; synovial sarcoma
 Most synovial sarcomas are: monophasic
 Immunohistochemistry for synovial sarcoma: + for keratin and
epithelial membrane antigen (EMA)
 + for keratin and epithelial membrane antigen (EMA) : synovial
sarcoma.

Old test tq info:

 the can inhibit methotrexate activey and is used as a rescue agent
in mtx toxicity: luecorvin
 what is a concern about mtx: concurrent use of NSAIDS deceases
the clearance of MTX so toxicity can dev rapidly: (death from
severe bone marrow suppression) has been linked to ibuprofen/mtx
use. (ibuprofen same as motrin and advil)
 causes undersecretion of uric acid:
o dehydration,
o ketosis,
o renal abnor.
o Drug: directics, low dose ASA,
o toxins such as ethanol, or lead,
o hypothyroidism
 causes overproduction of uric acid
o ethanol
o def of HGPRT or G6PD
o superactive PRPP synthetase
o Myeloproliferative disorders
o Psoriasis
 Clubbing of digits: usually occurs in pts with bronchial carcinomas
but sometimes with pleural tumors, pulmonary metastes,
mediastinal Hodgkin dz, chronic lung inf, and chronic liver dz
 Complication of tnf alpha inhibitor injections: activation of recurrent
TB inf
 Albrights ass with: polyostotic bone lesions, endocrine dysfunctions,
acromegaly, cushion syndrome, hyperparathyroidism, pigmented
macules (café au lait spots) over butt, back and sacrum, Chinese
characters
 What does colchicine tx: attack of gout
 Sunburst or sun ray vs sunburnt:
o sunburst/ray: osteosarcoma (osteogenic sarcoma)
o sunburnt pattern: ewing sarcoma (which is a metastatic
tumor of bone)
 histological pattern hallmark in mosaic pattern: pagets dz
 best test to dx osteoporosis: DEXA scan
 osteopenia defined as BMD score of: btw -1.0 SD and -2.5 SD
below normal
 osteoporosis defined as BMD score of : less than -2.5 SD below
normal
 benign tumor of young persons, M>F and usually in LE including
small bones of foot. Pain with more sever at night. Xray shows
central radiolucent area (NIDUS) surrounded by reactive sclerotic
bone: osteoid osteoma
 crystals found in synovial fluid
o monosodium urate monoydrate: acute gout, tophaceous gout
o calcium pyrophosphate dihydrate: acute psueogout,
destructive arthropathy
o basic calcium phosphate: acute, subacute arthritis
o lipid: acute arthritis
o cholesterole: asymptomatic
 anca ass with
o wegeners granulomatous, microscopic polyangiitis, churg
strauss syndrome, drug induces vasculitis
 KIMMEL: best way to differentiate btw RA and OA; Hx and PE
 Bizarre pleomorphic cells with hyperchromatic irregular nuclei and
abundant mitoses: osteosarcoma (osteogenic sarcoma)
 Formation of osteoid and bone in this highly malignant tumor:
soteosarcoma (osteogenic sarcoma)
 Inhibits IL1 AND IL2 and don’t drink grapefruit juice: cyclosporine
(Sandimmune, neoral)
 Pulmonary hypertension and centromeric ab: limited cutaneous
sclerocerma (LcSSc)
 SCL-70: diffuse cutaneous scleroderma (DcSSc)
 This scleroderma has a good prognosis: localized scleroderma
 Boutonniere deformity : seen in RA with PIP hyperflexed and DIP
hyperextended
 Whats a major contraindication for infliximab (Remicade): MS
 Wegeners granulomatous constists of
o Nasal or oral inf (oral ulcers or bloody nasal drainage)
o Abnormal chest radiograph (nodules, fixed infiltrates, cavities
o Urinary sediment >5rbc/hpf or RBC casts
o Granulomatous inf on bx (in wall of artery or arteiole,
perivascular or extravascular)
 Churg strauss syndrome (allergic granulomatosis and angitits)
o Asthma
o Eosinophilia (>10% wbc
o Mononeuropathy or polyneuropathy
o Transitory pulmonary infiltrates
o Paranasal sinus abnormalities
o Bx with extravascular eosinophils
 Polymyalgia rheumatica
o Pt over 50 and usually white
o Muscle pain for at least 1 month in shoulders and pelvic girdle
o Severe morning stiffness and gelling
 TA or giant cell arteritis
o Over 50
o New HA
o Temporal artery abnormality (tender or decreased pulse)
o ELEVATED ESR> OR = 50MM/HR
 o Abnormal art bx with mononuclear cell infiltrate,
granulomatous inf usually with multinucleated giant cells.
 Polyarteritis nodosa
o Weight loss >4kg
o Livedo retularis
o Testicular pain or tendernous
o Myalgias, weakness, or leg tendernous
o Mono or polyneuropathy
o Diasoltic >90
o Elevated bun or creatinine
o Heb B virus
o Arteriographic abnormatily
o Bx of small or medium artery containing PAN
 In osteoporosis there is a loss of height and multilevel fx that can
lead to lumbar lordosis or kyphoscoliosis. What would you also find
to be elevated: IL1, IL6 and TNFalpha, rank, and rankL, osteoclast
activity.
o You would find decreased: serum estrogen, response of
osteoprogenitor cells, osteoblast act, and Physical activity
 You might find secondary hyperparathyroidism in this: osteomalacia
due to low vit D. (undermineralized bone)
 Dry eyes and dry mouth: sjogrens syndrome
 Anti-SSA (ro) and anti SSb (la): sjogrens syndrome
 Rose Bengal staining/ schrimer test: sjogrens syndrome
 Lip bx: sjogrens syndrome
 Tx for eyes in sjogrens syndrome: restasis
 Tx for oral sx: Evoxac/ Salagen
 What should be started within 6 months of dx of RA: dmards
 How long to wait for preggo in lefunamide (Arava): 2 years
 How long to wait for mtx: 3-6 months
 Xray of knee showing medial joint space narrowing: OA
 Tx for PVNS or GCT: surgery
 Xray finding of thickened irregular and lobulated epiphyseal plate:
rickets (children vit d def)
 Rachitic rosary (swollen constocondral junctions of ribs) and pigeon
chest: rickets
 Bowing of arms and legs: rickets
 Osteopenic x ray pattern: osteomalacia
 Compression fx and decrease in bone thickness: osteomalacia
 Osteomalacia will also result in: secondary hyperparathyroidism
 Eburnation: oa
 In gout what is the tophus composed of: macrophages,
lymphocytes, fibroblasts, and foreign body giant cells
 Drug that inhibits lymphotoxin alpha: entanercept (Enbrel)
o Also inhibits tnf alpha and beta
 Can tx ra, juvenile arthritis, and ulcerative colitis
 Adverse: opportunistic inf and lymphomas
 Jones tq 100%
o Non inflammatory
 Leukocyte ct <3000/uL, few neutrophils
 Oa, traumatic arthritis, PVNS
o Inflammatory
 Leukocytes ct btw 3000-75,000/uL, 50% > neutrophils
 RA, sle, reiter syndrome, rheumatic fever, acute
cystic induced arthritis
o Purulent
Leukocyte ct >50,000, >90% neutrophils

 Bac, fungal, tuberculous joints
o Hemorrhagic
 Traumatic, PVNS, synovial hemangiomas, hematological
disorders, thrombocytopenia, anticoagulant therapy

 Inhibits uric acid formatin by inhibiting xanthine oxidase for tx of
gout: allopurinol
 Non purine xanthine oxidase inhibitor for gout: febuxostat
 Breaks down uric acid in gout: uricase
 To tx attack in gout: colchicine, nsaids, steroids
 What are the uricosuric drugs for gout: Probenecid/ sulfinpyrazone
 Osteitis deformans: pagets dz
 Brown tumors: osteitis fibrosa cystica
 Osteoclastoma: spindle shaped cells (aka giant cell tumor of bone)
 Sickle cell: salmonella
 Late adolescence and young adulthood: s. aureus
 Older children and adults: s. aureas
 Methotrexate can cause cirrosis
 Anti-jo-1: polymyositis/ dermatomyositis
 Anti ds dna: sle
 Anti-ribonuclear antibodies: (anti-RNP)::: mixed connective tissue
dz (MCTD)
 Shaft of a long bone: diaphysis
 Where does the ewing sarcoma originate in the bone: medullary
cavity
 Which form of scleroderma is ass with pulmonary hypertension:
limited cutaneous scleroderma
 Anti-phospholipid antibody syndrome: cardiolipin abs: ass with
abortions
 Round hyperchromatic cells with little cytoplasm and virtually no
stroma: ewing sarcoma
 Defect in intercellular matrix: mutation in gene ATP2A2 on chrome
12: darrier dz aka keratosis follicularis
 Onycholysis: psoriasis
 Acanthosis with elongated rete ridges: psoriasis
 What are munroe abscesses: neutrophils in psoriaais
 What atb is involved in pemphigus vulgaris: IgG
 Which skin disorder is ass with mg and lupus: pemphigus vulgaris
 Linear BMZ deposits of IgG and complement: bullous pemphigoid
trif
 what are the dual roles of articular cartilage hyaline type at ends of
bone:
o friction free movements
o absorption of shock and weight
 layers of the synovium
o macrophages with lysosomal enz and dense bodies
o cells secrete hyaluronic acid
 the synovial fluid controls:
o diffusion
o ingestion
o secretion of hyaluronate, immunoglobulins, and lysosomal enz
o lubrication by secretion of glycoproteins
 what is the main source of nutrients for articular cartilage since it
lacks blood supply: synovial fluid
 elastic and high tensile strength: hyaline cartilage
 what is hyaline cartilage made up off
o type 2 collage fibers: tensile strength and transmits vertical
load
o water and proteoglycans for: turgor and elasticity and limit
friction
o chondrocytes: syn matrix and digest matrix
 layers of chondrocytes from superficial to deep
o tangential or gliding:
 elongated, flattened and parrell
o transitional:
 chondrocytes are larger, ovoid and more randomly
distributed
o radial zone:
  small and arranged in short columns like in the
epiphyseal plate
o calcified zone: Deepest
 small chondrocytes and heavily calcified matrix

 what controls matrix turnover: chondrocytes
 cytokines/growth factors needed for osteoclast differentiation
o IL1, IL3, IL6, IL11, TNF, GM-CSF, M-CSF
 Stimulate osteoclast progenitor cells
 Participate in Paracrine system in which osteoblast and
marrow stromal cells play central role
 Mediators of the paracrine system
o Rank
o rankL
o osteoprotegerin
 what types of cells in rank mostly on: macrophage/monocytic
lineage such as pre-osteoclasts
 what types of cells is rankL on: cell membranes of osteoblasts and
marrow stromal cells

 what initiates osteoclastogenesis: rank binding to rankL
 what inhibits osteoclastogenesis: OPG by acting as a decoy that
binds RankL.
 Ehler-danlos, marfans or osteogenesis inperfecta: secondary Oa
 Achondroplasia, congenital hip dysplasia: secondary Oa
 Macro trauma or repeated microtrauma: secondary Oa
 Fibrillation (flaking: oa
 Subchondral bone cysts: oa
 Joint mice: oa
 Pain and guarding of the joint: synovial inflammation in oa
 Dull aching pain in oa: when full thickness cracks or erosions in
articular cartilage allow joint fluid to be forced through subchondral
bone plate
 Pain and bracing of the joint: capsular inflammation
 Non suppurative inflammatory and proliferative synovitis: RA
 What forms the Rf factor: B cells
 What activates the macrophages to produce cytokines in RA: t cells
 What activates neutrophils: IC and complement
 What are the mediators of inflam and destruction that are produced
by the neutrophils: prastaglandins, leukotrienes, lysosomal enz,
and ROS
 What produces RankL: t cells and synovial fibroblasts
 Is there genetics in RA: yes: HLA-DR
 Pannus consist of: inflammatory cells, granulation tissue, and
fibroblasts
 Extra articular involvement in RA
o subQ rheumatoid nodules
o vasculitis and inf lesions in pericardium, myocardium, lungs,
pleura, peripheral nerves, and eyes
o systemic amyloidosis in some pts
o lab finding of hypergammaglobulinemia: RA
 ass with gout:
o chronic hemolysis, polycythemia, leukemia, lymphoma,
diuretics, aspirin, nicotinic acid
o
 gauchers dz, sle, or osteo radio necrosis after radiation: avascular
necrosis etiology
 pale infarct or sequestrum and separtaiton of overlying cartilage
from bone: avascular necrosis morphology
 avascular necrosis of the femoral head in children: legg calve
Perthes dz
JONES lymphoid neoplasm’s:
 Part of LN where primary or secondary follicle (germinal center:
mantel zone) is: cortex
 Part of LN where sinuses are located: medulla
 Def: LN undergo reactive changes whenever they are challenged by
microbiologic agents, cell debris, or foreign matter introduced into
wounds or into circulation: lymphadenitis (NOT THE SAME AS
LYMPHADENOPATHY)
 Acute or chronic no specific lymphadenitis:
o Nodes are swollen, grey-red and engorged: acute
o Distention of capsule causes tendernous to touch: acute
o Which one has three types: chronic
 What are the three diff morphological alterations in
chronic non specific lymphadenitis:
 1. Follicular hyperplasia
 2. Paracortical lymphoid hyperplasia
 3. Reticular hyperplasia.
 What are the etiological factors in lymphoid neoplasm’s:
o Chromosomal translocations and oncogenes
o Inherited genetic factors
o Viruses
o Env agentes
o Iatrogenic
 Whats the difference in leukemia and lymphoma
o Leukemia: use for lymphoid neoplasm’s presenting with
WIDESPREAD involvement of the bone marrow, usually with
presence of large number of tumor cells in the peripheral
blood.
o Lymphoma: used to decribe proliferations as DISCRETE tissue
masses.
 What does the REAL chassification system use as the most
important criteria: immunophenotype
o Also: genetic aberrations
 Precursor b cell neoplasm: neoplasm of immature b cell
 Peripheral b cell neoplasm: neoplasm of Mature b cells
 Precurso t cell neoplasm: neoplasm of immature t cell
 Peripheral t cell and NK cell neoplasm: neoplasm’s of mature t/NK
cells.
 Neoplasm’s of reed Sternberg cells and variant: hodgkins lympoma

 What are the vast majority of lympoid neoplasm’s (80-85%): of b

cell origin
 Nodular sclerosis and mixed cellularity seen in : V. Hodgkin
lymphoma
 What is ALL: acute lymphoblastic leukemia/lymphoma
 Most occur in kids under 15, 2x as common in whites, and more
frequent in boys than girls, and 85% are of precursor b cell tumors:
ALL
 Abrupt stormy onset, bone pain and tendernous, generalized
lymphadenopathy, splenomegaly, or hepatomegaly and cns
manifestions: ALL
 Lymphoblasts with scant cytoplasm and nuclear chromatin is
delicate and finely stippled: ALL
 TdD is positive in >95%: ALL
blasts almosts always express the pan b cell molecule CD19/CD10:
ALL
 How is early and late ALL distinguished: early doesn’t have
cytoplasmic IgM heavy chain ( mu chain)
 35% or > # of blasts in the bone marrow is essential for dx: ALL
 POOR prognosis Markers in ALL
o Age <2
o Presentation in adolescence or adulthood
o Peripheral blood blasts counts greater than 100K,
o Cytogenic aberrations such as t(9;22) (****the
PHILADELPHIA CHROMOSOME)
 Favorable prognosis markers in ALL:
o Onset of dz between age of 2-10
o Low white count
o An early pre-B phenotype
 The dx criteria is an absolute lymphocyte count > what ????? in
chronic lymphocytic leukemia/small lymphocytic lymphoma: >4000
per mm (cubed)
 Most pts present at ages over 50 (median age is 60): chronic
lymphocytic leukemia/small lymphocytic lymphoma
 Gender pref in chronic lymphocytic leukemia/small lymphocytic
lymphoma: M 2x
 LN architecture is diffusely EFFACED by predominant pop of small
lympocytes 6-12 micro meters in diameter containing round to
slightly irregular neuclei with condensed chromatin and scan
cytoplasm: chronic lymphocytic leukemia/small lymphocytic
lymphoma
 Smudge cells: chronic lymphocytic leukemia/small lymphocytic
lymphoma
 B cel markers in chronic lymphocytic leukemia/small lymphocytic
lymphoma: CD19, CD20, CD23, CD5
 Of the above which one is the t cell marker that is expressed on
only a small subset of normal b cells in chronic lymphocytic
leukemia/small lymphocytic lymphoma: CD5
 Diffuse large b cell lymphoma transformation: Richter syndrome:
under chronic lymphocytic leukemia/small lymphocytic lymphoma
 Heterogeneous group of tumor that constitute 20% of all NHL and
60%-70% of aggressive lymphoid neoplasm’s: diffuse large b cell
lymphoma
 What is the median age for diffuse large b cell lymphoma: 60
 This is fatal but easily treatable: diffuse large b cell lymphoma
 Large cell (centroblastic) size (usually 4-5 x the diameter of a small
lymphocyte) and a diffuse pattern of growth: diffuse large b cell
lymphoma
 What are the markers on b cell tumors in diffuse large b cell
lymphoma: CD19, CD20
 Variable expression of germinal center markers CD10, and BCL6
and most have surface Ig, and all are negative for TdT: diffuse
large b cell lymphoma
 African (endemic) Burkitt lymphoma
 Sporadic (nonendemic) Burkitt lympoma
 Faintly basophilic or amphophilic cytoplasm: burkitts lymphoma
 Mitotic figures are numerous and evidence of APOPTOSIS: burkitts
lymphoma
 Starry sky pattern: Burkitts lymphoma
 Tumor cells are mature b cells expressing surgace IgM, monotypic
kappa or lambda light chain, CD19 CD20, CD10, and BCL6: Burkitts
lymphoma
 All forms of burkitts lymphoma are ass with the translocation of
the :c-myc gene on chromosome 8
 What age does most Burkitts lymphoma affect: children and young
adults
 Mass involving the mandible, abdominal viscera particularly the
kidney, ovaries, and adrenal glands: Burkitts lymphoma (endemic
form)
 Most often presents as ab mass involving ileocecum and
peritoneum: Burkitts lymphoma (sporadic form)
 Prognosis of BL: good when tx with high dose chemotheraphy for
short term
 Another name for multiple myeloma: plasma cell myeloma
 The most imp and most common symptomatic monoclonal
gammopathy: multiple myeloma
 Gender preg in multiple myeloma: men
 High serum levels of IL-6 are ass with poor prognosis; multiple
myeloma
 Most often affects the verterbral column: multiple myeloma
 The bone lesions appear as punched out defects 1-4 cm on xray:
multiple myeloma
 Flame cells: multiple myeloma
 Mott cells: multiple blue grapelike cytoplasmic droplets and cells
containing variety of other inclusions such as fibrils, crystallien
rods, and gobules sometimes russell bodies (cytoplasmic) or
dutcher bodies (nuclear): multiple myeloma
 Rouleaux formation caused by high levels of serum M proteins in :
multiple myeloma
 99% of pts with show increased levels of immunoglobulins in blod
and or light chains in urine known as BENCE JONES PROTEINS:
multiple myeloma
 most common serum monoclonal immunoglobulin (m protein) in
multiple myeloma: IgG
 prognosis of multiple myeloma: variable but generally poor
 high incidence in adults infected by HTLV-1: adult t cell
leukemia/lyphoma
 hypercalcemia: adult t cell leukemia/lyphoma
 cells have multilobulated nuclei called cloverleaf or flower cells;
adult t cell leukemia/lyphoma
 prognosis of adult t cell leukemia/lyphoma: death within 1 months
to 1 year even with aggressive chemo
 mycosis fungoides progresses to : Sezary syndrome
 different manifestations of a tumor of cd4 helpter t cells
characterized by a marked predilection to involve the skin.: mycosis
fungoides/Sezary syndrome
 shows three distinct stages of
o 1:inflammatory premycotic phase,
o 2. Plaque phase
o 3. Tumor phase
 Answer: mycocsis fungoides

 Manifests as generalized exfoliative erythroderma: Sezary
syndrome
o
o
 Neoplastic giant cells seen in HL: reed Sternberg cells
 What are reed Sternberg cells derived from in HL: from germinal
center or post germinal center b cells
 Most common form of malignancy in young adults with ave age of
dx of 32: HL
 Prognosis of HL: curable in most cases
 Nodular sclerosis pts usually present as: stage 1 or 2 M=F
 Mixed cellularity pts usually present as: state 3 or 4 M>F
 Lacunar cells: type of reed Sternberg cell seen in HL
 Mummification: HL
 Spread of HL: nodal dz firstsplenic dz hepatic finally marrow
and extranodal involvement
 Most common form of HL: nodular sclerosis type
 Nodular type most common cell type: lacunar cell type
 Collagen bands: nodular type of HL
 CD15 CD30 and neg for CD45 and b cell and t cell markers: nodular
type of HL
 Which type of HL has strong ass with EBV: mixed cellularity type
 Which has best prognosis: nodular type
 Which one has night sweats and weight loss: mixed cellularity type
 Lytic lesions: Multiple myeloma
 Mott cell: multiple myeloma
\

HEME OLD TEST QUESTIONS

 Common translocation is the IgH locus t(8:14) but there may also
be t(2;8) or lambda (8;22) light chain locus: burkitts lymphoma
 Jaw involvement: endemic burkitts lymphoma
 Philadelpha chromosome: poor prognosis in ALL
 Majority of cells have terminal deoxynucelotideyltransferase (TdT)
(+ in >95%) of cases): ALL
 Absolute lymphocytic count >4000 per mm cubed: CLL
 Intranasal cyanocobalamin for : tx fof vit b12 def
 Inhibits dehydroooratic acid dehydrogenase: luflunamide (arava)
 Smudge cells: CLL
 In the anemia, RBC hemolysis results in
o Increased internal viscosity
o Hb aggregation
o Decreased cell water
o And precipitated hb as Heinz bodies:
 Answer: G6PD def
 What can cause G6PD def to unmasked:
o Exposure to some drugs: ex sulfonamide
o Inf
o Diabetic acidosis
o Neonatal period
o Ingestion of FAVA beans
 Fava: g6pd def
 Osmotic fragility: hereditary spherocytosis
 Whats the tx for hereditary spherocytosis/elliptocytosis:
splenectomy
 Defect in the RBC membrane results in these two disease:
hereditary spherocytosis and hereditary elliptocytosis
 Found in southern japan, west Africa, and Caribbean basin: adult T
cell leukemia/lymphoma
 Starry sky appearance: burkitts lymphoma
 In an indirect coombs test where is the ab: in the plasma
 In a direct coombs test, where is the ab: on the membrane
 Hematuria vs hemoglobinuria: in hematuaria the rbc is intact where
as in hemoglobinuria the rbc is lysed.
 Which one of the above is ass with uti or kidney problems:
hematuria.
 Which one of above is associated with hemolytic anemia:
hemoglobinuria

 Polycythemia aka: erythrocytosis
 In relative polycythemia due to dehydration, hct levels are: high
 In relative polycythemia due to spurious, hct levels are low
 What are the macrocytic anemia: (mcv <80)
o Find those small cells henry
 Fe def or pb poisoning
 Thalassemias
 Sideroblastic (80%)
 Chronic dz
 Hemoglobinopathy
 What is the purpose of a reticulocyte count: it shows how fast rbc
cells called reticulocytes are being made and put into the blood.
o The reticulocyte count can rise in dz where there is a loss of
blood or premature destruction of rbc such as in : hemolytic
anemia

 Petechia: platelets
 What are the four d’s of quantitative thrombocytopenia:
o Increased destruction
 o Decreased production
o Dilution
o Distribution

 What are the myeloid cells:
o Basophils
o Rbc
o Eosinophils
o Monocytes
o Platelets
o
 What are the lymphoid cells
o Lymphocytes
o Lymph nodes
o Extranodal tissue

 Myeloproliferative disorders affect what age: adults
 Myeloproliferative disorders are disorders of: stem cells
 What does the peripheral smear show in Myeloproliferative
disorder: increased platelets
 What does the bone marrow show in myeloproliferative disorders:
HYPERcellularity

 Myeloproliferative disorders:
o Name the predominant phenotypes
 Chronic myeloid leukemia: granulocytes, basophils,
eosinophils
  Polycythemia vera: erythocytes
 Essential thrombocythemia myelofibrosis:
platelets/megakaryocytes
 Chronic myelomonocytic leukemia: monocytes

 Walenstroms macroglobulinemia: plasma cell disorder
 What are all the plasma cell disorders
o MM
o LIGHt/heavy chain dz
o Benign monoclonal gammopathy
o Waldenstroms macroglobulinemia
o Amyloidosis
o Plasmacytoma
o Plasma cell leukemia
o Monoclonal gammopathy of undertermined significance

 How to evaluate coagulopathy
o Whats the best way: Hx and PE
o CBC with smear
o PT: prothrombin time
 Extrinsic pathway: factors 2, 5, 7, 10 and fibrinogen
o INR: international normalized ratio
 Ratio of pts PT to normal PT
o PTT: partial thromboplastin time
 Factors: 8,9,11,12, and rarely von villebrands factor
 o Platelet count

 What are the inherited defects in anti-clot enz ass with
hypercoagulable states: protein C and protein S def.
o Also (HIT ab)

 What are the clinical manifestations of anemia (TQ 100%)
o Fatique/weakness
o Dizziness/ syncope
o Intermittent claudication
o Muscle cramps at night
o HA/ lightheadedness
o Angina
o Tinnitis

 What is on a peripheral smear in hemolytic anemia: spherocytes
 TQ 100%: general lab features of hemolytic anemia
o Low Hgb/Hct
o HIGH reticulocyte count
o Increased LDH
o DECREASED haptoglobulin
o Increased bilirubin
 What are the SPECIFIC lab features of hemolytic anemia
o Schistocytes/poikliocytes/spherocytes/ovalcytes/sickle cells
o Hemoglobin electrophoresis
 o Hemoglobinemia/hemoglobinuria
o Osmotic fragility
o DAT/IAT/ eluates
o G6PD level
o ETC

 Priapism: perpetual erection seen in sickle cell
 What is the most commonly used anti-sickling agent tx for sickle
cell: hydroxyurea
 Which one do you use an RBC exchange: sickle cell anemia

 Beta thal minor: thal triait: 1 gene abnormal: normal hb
 Beta thal intermeida: 2 genes abnormal but some beta globin
produced: mild to moderate anemia
 Beta thal major: 2 genes abnormal and little beta globin produced:
severe anemia: high Hgb F -1 to 5% HgbA

 Alpha thalassemias
o Silent carrier: 1 missing gene, normal Hgb: no dz
o Alpha thalassemia trait/alpha thalassemia minor: 2 missing
genes: mild anemia
o Hemoglobin H: three missing genes mod to severe anemia
o ***** it takes three lines to make an H)
o alpha thalassemia major or hydrops Fetalis: all Four genes
missing: death before or shortly after birth

 tx is none except transfusion and IRON Chealation with BAL:
thalassemias
 Heinz bodies: eating fava beans in G6PD def
 Whats does WAIHA stand for: warm autoimmune hemolyic anemia
 Is warm ab auto or Alloimmune: autoimmune
 Is HDN auto or allo: Alloimmune
 Penicillin: Alloimmune
 Lupus is ass with : autoimmune
 What are the lab values in hemolytic anemia
o Anemia
o In vivio hemolysis
o +DAT
o + IAT
o eluate to ID antibody
o reticulocytosis
o spherocytes
o splenomegaly
o
 what is the antibody in WAIHA: IgG
 TTP is a type of : acquired hemolyic anemia
 What is the pentad of TTP
o Microangiopathic hemolytic anemia
o Fever
o Renal failure
o Mental status changes
o Thrombocytopenia

 Adempts 13 def: TTP
 Tx is plasma exchange: TTP
 What is the disorder in AA: the pluripotent stem cell
 What are the hallmarks of AA:
o Requires both
 Pancytopenia
 Acellular bone marrow filled with fat

 What is the cuase of AA: stem cell injury

 What are you looking for on AA bone marrow bx: spicules with
empty fatty spaces
 What is the total lab eval in AA
o Pancytopenia
o Acellular or hypocellular BM
o Decreased CFU-GM
o Decreased BFU-E
o Decreased Long term culture initiating cells to les than 1%
o Flow cytometry rules out ALL or hairy cell leukemia

 What rules out ALL and hairy cell leukemia in AA: flow cytometry
 Tx for AA: bone marrow transplant
 What test to order to r/o myelodysplastic syndrome: cell
morphololgy/ cytogenetics, cell surface markers
 Test to r/o PNH: + sucrose hemolysis test/ abnormal CD59
 ALL: cell surface markers/marrow
 Hairy cell leukemia: cell surface markers and special stains
Kimmel last days before exam
 Paste features seen in what: generalized discoid lupus (DLE)
 What does paste stand for
o Follicular Pluggin
o Epidermal Atrophy
o Scale
o Tenangiectasia
o Erythema
 Which type of cutaneous lupus has the greatest risk for SLE (70%):
acute LE (ALE)
 What are the possible involved areas in systemic lupus (SLE): old
TQ
o Skin, joints, kindneys, lungs, brain, heart, blood forming
organs

 Photosensitivity and malar rash: SLE
 What are the hematological levels in SLE:
o Hemolytic anemia
o Leukopenia: <4K
o Lymphopenia: <1.5k
o Thrombocytopenia: <100K
 Pt with the following: ds dna, sm, antiphospholid with false + VDRL,
cariolipin ab, and lupus anticoagulant: SLE
 If you get a neg ana for sle: much less likely that you have SLE
 If you get a + ana what is next step: further testing

 What are the major specific serologies for SLE
 o DS DNA
o ENA
o ACL abs
 What is the sensitive but not specific serology for SLE: ANA

 What do you tx the skin in sle with:
o Antimalarials
o Steroids
o Dmards (anti metabolites, MTX, Leflunomide, MMF, aza)
o Cytotoxic: CSA, CTX)
 What do you tx the joints in SLE
o NSAIDS: only if no kidney dz
o Anti malarials
o Steroids
o Dmards
o Biologicals
 Major inter organ tx in sle:
o High dose steroids
o +/- cytotoxic agent

 for SLE kidney tx
o focal proliferative : steroids
o diffuse proliferative: steroids + Cytoxan/MMF or BM
transplant
o membranous: steroids +/- IMURAN/ cyclosporin A

 what do you tx the thrombocytopenia in SLE with if its
o mild: Plaquenil or danazol
o severe: prednisone (others in notes)

 what is usually not affected in drug induced lupus: major internal
oragn dz or DNA abs
 TQ: what are the drugs that can cause drug induced lupus
o P(2x)HIC Q
 Procainamide
 penicillamine
 Hydralazine
 Isoniazid
 Chlorpromazine
 Quinidine

 Increased PTT time not corrected by mixing study:

anti=phospholipid ab syndrome
 What is the tx of the above: anticoagulant
 Cardiolipin ab: anti-phospholipid ab syndrome
 What things can be false + in pt takig anticoagulants in anti-
phospholipid syndrome: lupus anticoaguland and PTT time
 Tx in anti phosopholid ab syndrome for pts with previous
thromobotic episodes: Warfarin with INR

 Morphea: localized scleroderma
 Crest: limited cutaneous scleroderma
  Pulmonary HTN: limited cutaneous scleroderma
 SCL-70: diffuse cutaneous scleroderma
 Cancer risk such as lung cancer in: scleroderma
 Manifestions such as raynauds, digital ulcers, calcinosis, MSK, GI,
renal, pulm, and cardiac : scleroderma

 Tendon inflammation/ friction rub/tenosynovitis in: PSS
 Ace inhibitor or vasodilator: tx option for PSS

 Name the dz: symmetric proximal muscle weakness, elevated
Muscle enz such as (CPK, aldolase, and transaminase, LDH),
myopathic EMG abnormalities, typical changes in muscle bx,
heliotropic rash: polymyositis and dermatomyositis
 What are the ab ass with dermatomyositis: JO-1, PM-1
 If you have myositic what tests should you have: age related
malignancy eval on first dx + CXR and CT scan abdomen/pelvis and
yearly thereafter
 Antibody for Mixed CT dz: anti ribonuclear *anti RNP

Ditcheks lectures
 Best for spatial resolution: plain radiograph
 Initial for trauma: plain radiograph
 Good for pre-opt: CT
 High contrast resolution:MRI
 No radiation and used to differ btw cystic and solid lesion:
Ultrasound
 Avascular necrosis (AVN): MRI
 Achilles tendon: ultrasound
Foreign body such as WOOD: Ultrasound
 Advantage is a head to toe scan: bone scan
 What is bone densitometry (DEXA) used for : osteoporosis
 T scoring:
o Normal: within 1
o Osteopenia: 1-2.5 below the young normal bone density
o Osteoporosis: >2.5 below
 Def of comminuted: >2 fragments
 Impactation: bone teloscopes into itself
 Pathological fx: through abnormal bone such as in pagets dz
 Lipohemarthrosis: fat and blood in joints: means there is a crack in
the blood and the BM fat and blood leaked out.
 Pain and tendernous on outstretched hand: scaphoid fx
 For knee trauma to visualize ligaments, tendons, and cartilage such
as PCL or meniscus tear: MRI
 Best for comminated fx: CT
 Ped fx vs adult: peds bones more water content so they bend
before breaking. Can result in bent bone
 Buckling on xray: torus fx
 Bowing fx: from microfractures
 Look at salter harris classifications: TQ 100%
 Exits through metaphysis: type II
 Exits through epiphysis: type 3
 Exits through both: type 4
 Fat pads in elbow: elbow fx
o You should never see a posterior fat pad. Maybe you could
see a anteriorly normally
 Posterior rib fx: child abuse
 Bucket handle or corner fxs: child abuse
 Multiple fx in varying stages of healing
 What are the ABCDS of arthritis
 o Alignment
o Bone mineralization
o Cartilage (joint space)
o Distribution
o Soft tissues
 Ulnar deviation and periarticular osteopenia: RA
 Joint space narrowing and erosions: RA
 Young males, proximal large joints (spine/SI joint), and bamboo
spine: ankylosing spondylitis
 Non erosive, subluxations, distribution similar to RA (MCP) joints:
SLE
 First MTP: podagra: gout
 Distal joints in hands and erosive and productive and SI joints/spine
involved: psoriatic arthritis
 Which is the only one without erosions: SLE
 TQ: signs of non aggressive lesions
o Narrow zone of transtition
o Benign patterns of periosteal rxn
 Thick
 Wavy
 Dense
o No cortical destruction
 TQ: signs of aggressive
o Wide zone of transition
o Permeative or moth eaten lytic pattern
o Aggressive periosteal rxn
 Sunburst, onion skin, codmans triangle
 o Cortical destruction
 Monostotic: one location
 Which test to see monostotic vs polyostotic or increased bone
turnover: bone scan
 Soft tissue mass: MRI
 Rupture of rotator cuff: MRI
 Metastatic breast cancer: CT scan or MRI
 Evaluation pros for nuclear med for osteomyeltitis
o High sensititivity (bone turnover)
o Detects earlier than plain radiograph
o No artifact from prosthesis (unlike mri)
 Cons:
o Low specific
o Low resolution
o No info on soft tissues such as abscesses
 Best for osteomyeltits marrow edema: MRI

Weisberg
 Of the hodgkins which is more common: NHL
 Viral genomic material is found in 50% of HL tumors: EBV
 What is the gender preg for HL: Male
 Is HL genetic: Yes
 Painless lymphadenopathy, granulocytic leukocytosis with
eosinophila: HL
 What is the alkaline phosphate level in HL pts: elevated
 What are the B symptoms: Fever, night sweats, weight loss more
than 10% total body weight
 What cell origin is HL: B lymphocytic origin
 Immunoperoxidase rxn in HL will detect what two antigens helpful
in dx HL: Leu-M1 (CD15) and Ki-1 (CD30)
 What is the most common type of HL: nodular: also this is the best
prognosis also
o COTSWORLD STAGING CLASSIFICATION
 Waldeyers rings: stage I
 2 or > and on same side of diaphragm: II
 what is the consistency in HL lymph nodes: rubbery
 what does X mean: bulky dz
o greater than 1/3 widening of mediastinum
o greater than 10 cm dimension of nodal mass
 E stands for : involvement of a single extranodal site
 Unfavorable prognosis factors of localized dz (stage I or II)
o Elevated ESR >30
o Histology: mixed cellularity or lymphocyte deplated
o Bulk dz: ratio of mediastinal mass to thoracid diameter >1:3
o 4 sites on same side of diaphragm
 what are some unfavorable prognosis factors in advanced dz
o serum albumin <4g
o Hb <10.5
o Male
o Stage IV
o WBC >15,000
o Age >26
o Lymphocyte count <600 or <8% of white count

 what makes pain worse in HL when drinking it: alcohol
 tx for stage IA or IIA
o radiation and often preceded by brief chemo
 what are the long term effects of radiation therapy
o HYPOthyroidism
o Radiation fibrosis
o Effusions
o Late occurring solid tumors in field of irradiation
o Sterility
 What is the tx for advanced age dz of HL (IIIA, IIIB, or IV)
o Combination chemotherapy as primary therapy with the
possibility of radiation for residual masses
 In the above what does combonation therapy consists of
o MOPP
 Mustargen
 Oncovin
 Prednisone
 Procarbazine
o Or ABVD---------------->>>> first line now
 Adriamycin
 Bleomycin
 Vinblastine
 Dacarbazine
 What are the drugs in the HIGH dose therapy options for pt that
relapse
 o CBV
 Cyclophosphamide
 Carmustine
 Etoposide
o BEAM
 Carmustine
 Etoposide
 Cytarabine
 Melophalan

NON HODGKIN LYMPHOMA
 What are the cell types involved: B cell, T cell or NK lymphocytes
 What is the tx of choice for NHL: Rituxan
 Low grade lymphomas in NHL are: seldom curable
o But tx can often be delayed with no consequences
 TQ: what are the infection agents knows to cause NHL
o EBV
o HTLV1
o H. pylori
 What are the physical or chemicals ass with NHL
o Diphenylhydantoin
o Dioxin
o Solvents
o Radiation
 o Prior chemo
o Prior radiation
 Inherited immunodeficiency dz’s
o Klinefelters
o Chediak higashi syndrome
o Ataxia telangictasia syndrome
o Wiscott Aldrich syndrome
o Common variable immunodef syn
 Acquired immunodeficiency dz
o Iatrogenic immunosuppression
o Acquired immunodeficiency syn (HIV)
o Acquired hypogammaglobulinemia
 Autoimmune dz
o Sjogrens
o Non tropical sprue
o RA
o SLE
 What are the mature (peripheral) b cell neoplasm’s
o B cell lymphocytic leukemia/small lymphocytic lymphoma
o Follicle center lymphoma, follicular (most common)
o Mantel cell lymphoma
o Diffuse lare cell b cell lymphoma
o Burkitts lymphoma/Burkitt cell leukemia
 What are the precursor t cell neoplasm’s
 o Precursor t lymphoblastic lymphoma/leukemia
 What are the mature (peripheral) t cell and NK cell neoplasm’s
o Mycosis fungoides/Sezary syndrome

 What is the most common site of MALT lymphomas: stomach
 What are the causes of gastric MALTomas: H pylori thus (TQ) you
can tx MALT with an antibiotic

 Whats the t cell ones: mycosis fungoides and Sezary syndrome
 Localized vs advanced
o Localized (must have all three)
1. Ann arbor I or II
2. No tumors >10cm
 3> no B symptoms
o advanced (May include any of the three)
 1. Ann arbor III or IV
 2. Tumors >10cm (bulky dz)
 3. B symptoms present
 explain the IPI pts
o APELS
 Age over 60: 1 pt
 Performance exam > or = 2: 1 pt
 Extranodal sites >1: 1 pt
 LDH above ave: 1 pt
 Stage III or IV: 1 pt

 What is the difference in the FLIPI index
o The number of LN involvement has to be 4 and HB <12.

 Tx for indolent lymphomas
o Alkylating agents: chlorambucil, cyclophosphamide,
bendamustine
o Anthracyclines: doxorubicin
o Purine analogues: fludarabine
o RUTUXAN: chimeric ab with anti CD20 activity
o

 What are some signs of progression to aggressive lymphoma in NHL
o New or worsening B symptoms
o Facial or UE swelling
o Ab pain
o Jaundice
o Constipation
o Bowell obstruction
o Painful, rapidly enlarging LN
o Tender splenomegaly
o Superior vena cava syndrome
o LE edema
o Thrombocytopenia
o Granulocytopenia
o Rising serum creatinine
 o Hyperbilirubinemia
o Hydronephrosis on CT
 What are the chemotherapy tx for NHL
o Single alkylating agent
 Chlorambucil, Cytoxan
 CHOP, CVP
 New agents: bortezomide, bendamstine,
radionucleotides

Intravascular hemolysis anemias

o Hemolytic anemia
 Hemoglobinemia and hemoglobinuria
 Jaundice

FEMALE preference
 RA
 Juxtacortical osteosarcoma
 Giant cell tumor of bone (osteoclastoma)

08/09/2010
08/09/2010