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HEMATOLOGY

This document discusses the components and functions of blood plasma and formed elements such as red blood cells, white blood cells and platelets. It covers where these elements are produced, their characteristics and functions. The document also describes the stages of hemostasis and fibrinolysis.

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0% found this document useful (0 votes)
35 views23 pages

HEMATOLOGY

This document discusses the components and functions of blood plasma and formed elements such as red blood cells, white blood cells and platelets. It covers where these elements are produced, their characteristics and functions. The document also describes the stages of hemostasis and fibrinolysis.

Uploaded by

jsreyes.402
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as DOCX, PDF, TXT or read online on Scribd
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HEMATOLOGY

Plasma Components
Section I: Anatomy and Physiology Water Acts as solvent and suspension for
other blood components
Chapter I: Blood Function and Composition
Proteins Albumins
The functions of blood can be determined according to its - Oncotic pressure
components. Globulins
- Transport proteins (alpha &
beta) such as thyroxin-
binding globulins and
transferrin
- Antibodies (gamma)
Fibrinogen
- Clot formation
Other solutes Ions
- Osmotic pressure (Na+ & Cl-)
Nutrients
- Energy source and cellular
building blocks (glucose,
amino acids)
Gases
- Aerobic respiration (O2 &
CO2)
Waste products
- Urea, ammonia, bilirubin
Regulatory substances
- Catalyze chemical reactions
(enzymes)
- Stimulate/inhibit bodily
functions (hormones)
Formed Elements
Platelets Blood clot formation
WBCs Infection control and pathogen
inhibition
RBCs Oxygen transportation

Chapter II: Where Formed Elements are Produced

Production of RBCs, WBCs, and Platelets is termed as


“hematopoiesis”. They start as stem cells (hemocytoblasts) in the
red bone marrow. It is produced by flat bones (pelvis, ribs,
vertebrae, sternum, skull), epiphysis of long bones (during
childhood) and extramedullary organs (liver and spleen).

Chapter III: Formed Elements and their Characteristics

Red Blood Cells

- Biconcave in shape (greater surface area, flexibility)


- Thin membrane (for easy diffusion of oxygen)
- Main composed of hemoglobin (95% of mass) which not
only transports oxygen but can bind to H+ in venous blood
- Unnucleated (cannot divide)
- 90-120 days lifespan
- Susceptible to carbon monoxide binding (210x more than
oxygen)
- Needs B9 and B12 (animal origin, needs intrinsic factor to be
absorbed in the distal ileum) for DNA synthesis
- Needs iron for heme production
- Production is triggered with hypoxia (EPO released by
kidneys) Agranulocytes lymphocytes
- Increase reticulocyte count is usually triggered by acute - pathogen inhibition
blood loss - T-cells: cell-mediated immunity (CD4,
- Pregnant women needs up to 30mg of iron daily, 12mg for CD8), acts against foreign tissues and
men, and 15mg for menstruating women tumor cells
- Iron is stored is ferritin and bind to transferrin to be - B-cells: humoral/adaptive immunity
via antibody production
transported into normoblasts (erythrocyte precursor)
(immunoglobulins)
- RBC iron is usually recycled and some hemoglobin forms
- Plasma cells: most mature form of B-
bilirubin secreted in the bile to be excreted cell, secretes Ig
White Blood Cells - NK cells: specializes in virus and
cancer cells
- Nucleated (do not undergo mitosis) - Produced in marrow (majority are B-
- Ameboid movement cells) and thymus (T-cells) and
- Production triggered by infection - Matures in lymphoid tissues such as
- Removes debris via phagocytosis spleen and lymph nodes
- “Left shift: means more immature than mature monocytes
Agranulocytes: lymphocytes, monocytes (becomes - becomes macrophages and presents
antigen to lymphocytes
macrophages and presents antigen to lymphocytes)
- specialized against fungus and
Types viruses
Granulocytes neutrophils - immune surveillance
- Phagocytosis, 1st responders to - gives rise to Kupffer cells (liver),
bacterial infection peritoneal macrophages, and
- Stains pinkish-purplish alveolar macrophages (lifespan is
eosinophils months-years)
- allergic reactions (neutralizes
histamine)
- stains red
Platelets
- phagocytosis of parasites
basophils - Fragments of megakaryocytes
- histamine and heparin secretion - Facilitates hemostasis
- stains blue - Serum is plasma without clotting factors
Stages of Hemostasis Platelet-derived growth factors
Platelet inactivators:
- Nitric oxide and PGI2 (secreted by endothelial cells) VEGF
- Antithrombin III (bound to heparin sulfate in endothelial 5. Fibrinolysis Plasminogen converted into plasmin
wall)
- Protein-C (activated by thrombin-thrombomodulin factor) D-dimer as byproducts
1. Vascular Endothelin from damaged endothelial Notable medications Bigatran
spasm cells and TxA2 + serotonin from platelets - Blocks activation of CF II
induces spasm when bound to muscle Clopidogrel
cells - Inhibits ADP
Aspirin
Myogenic reflex - Blocks TxA2
2. Platelet plug Platelet adhesion to VWF Abciximab
formation - Blocks Gp2b/3a connection,
Platelet aggregation by fibrinogen thus inhibiting ase aggregation
receptors Warfarin
3. Coagulation Clotting factors activated forming fibrin - Inhibits production of Vit. K-
as the end product dependent clotting factors
Heparin
Affected by Vit. K (diet and gut bacteria - Potentiates antithrombin 3
that can be killed by antibiotics), calcium TPA
levels (CF 4), liver dysfunctions (CF IX, X, - Converts plasminogen into
VII, II plasmin

Intrinsic Pathway
- Starts w/ CF XII
- Measured by PTT time (25-35
secs)
Extrinsic Pathway
- Starts w/ CF III
- Measured by PT time (9-12 secs)

4. Clot retraction Platelet contraction


Chapter IV: Blood Grouping and Typing

Blood is categorized according to the type of antigen they have on


their surfaces. Consequently, antibodies in the plasma are opposite
to the surface antigens. When blood is mixed with a non-compatible
blood, agglutination (RBC clumping) and hemolysis occurs.

ABO Typing

Rhesus Factor

- Antibodies developed at first transfusion


- Rh-incompatibility common in hemolytic disease of
newborn (erythroblastosis fetalis) when the id Rh (-) and the
fetus is Rh (+).
- Rh (+) can receive Rh (-) but Rh (-) can’t receive Rh (+)

Crossmatching
- Blood of donor is mixed with the blood of the recipient to
test for agglutination

Chapter V: Normal Blood Values

Component Normal value


RBC 4.6-6.2 (male)
4.2-5.4 (females)
Hemoglobin 13-17 (male)
12-15 (female)
Hematocrit 40%-54% (male)
38%-48% (female)
WBC 4.5-11
- Basophils 0.5-1%
- Eosinophils 2-4%
- Neutrophils 60-70%
- Monocytes 3-8%
- Lymphocytes 20-25%
Platelet 200-450

Section II: Assessment

Chapter I: Physical Assessment


Chapter II: Health History

Chapter III: Diagnostic Evaluation

CBC Procedure
1. Blood via venipuncture
2. Peripheral smear on glass slide
3. Staining
4. Examination under microscope
PTT Measures integrity of intrinsic pathway Potential Risks:
- Bleeding
Normal: 25-35 secs - Infection
PT Measures integrity of extrinsic pathway Normal: semifluid state

Normal: 9-12 secs


International How fast your blood clots Section III: Therapeutic Approaches
normalized ratio Splenectomy Indications
Normal: 0.8-1.1 - Rapid RBC destruction
On blood-thinning meds: 2.0-3.0 is - Thrombocytopenia due
considered normal to sequestration
BMA and biopsy Aspiration from posterior iliac crest or
sternum Must be vaccinated for
pneumonia, influenza, and
Procedure meningococci prior to
1. Explain procedure, risks, and procedure (high risk for
alternatives infection)
2. May require anxiolytics Apheresis Separation of blood
3. Informed consent components via centrifuge
4. Skin is cleansed
5. Local anesthesia given *plasmapheresis
6. Skin may be punctured first *plateletpheresis
(bore is large) *leukapheresis
7. Introduce stylet then insert *erythrocytapheresis
needle *stem cell harvest
8. Expect sharp, brief pain Hematopoietic stem cell Provides longer remission
9. Aspirate 5 mL (deep breaths) transplant
10. Apply pressure then cover with Allogenic: from donor
sterile dressing
11. May ache for 1-2 days (warm Autologous: from self
tub baths, and mild analgesics Therapeutic phlebotomy Indications
prescribed, no rigorous activity - High hematocrit
and blood thinners for 1-2 days) - Hemochromatosis
*about 500 mL of blood
removed
Blood component therapy PRBC
- 70% hct
- Stored at 4C or 39.2F

Platelet
- Stored at room temp
- Lasts 5 days
- Gently agitated while
stored

Plasma
- Must be frozen
- Lasts 1 year
Special preparations Factor 8 concentrate
- For hemophilia A

Factor 9 concentrate
- For hemophilia B

Recombinant Factor 8
(Humate-P, Alphanate)
- VWF disease
- Hemophilia A

Plasma albumin
- Hypovolemic hock

Immune globulin
- IgG (for recurrent
bacterial infection
caused by leukemia)
- Iron supplements administered
- Advantage: lower risk for transfusion reactions

Pretransfusion Assessment

1. History
- Multigravida are higher risks for reactions
Procuring Blood and Blood Products - Previous reactions
- Interview to assess general health of donor - Cardiac, pulmonary, vascular disorders
- No upper age limit 2. Physical Assessment
- At least 50 kg for a standard 450 mL donation - Lung auscultation
- 8 weeks recovery before another donation - Edema and signs of heart failure
- Temp not exceeding 37.5C - Skin for rashes, ecchymoses, or petechiae
- Hgb at least 12.5 mg/dL - Eyes for icterus (jaundice)
- Directed donations: from relative
- If excessive bleeding occurs after donation, lie down with
head below the knees Transfusion Complications
Febrile non- Most common
Procedure hemolytic reaction Antibodies react to remaining WBCs in the
blood
1. Semi recumbent position
2. Area cleansed
Manifests as chills then fever.
3. Tourniquet applied
4. Venipuncture takes about 15 minutes Management :
5. Pressure applied for 2-3 minutes with arms elevated - Antipyretics given after the
6. Firm bandage applied transfusion, NOT BEFORE!
7. Food and fluids after. At least 15 mins rest Acute hemolytic Intravascular hemolysis due to ABO-
8. Avoid lifting, smoking, and alcohol for several hours reaction incompatibility

Autologous donation Manifests as fever, chills, low backpain,


- Blood from self chest tightness, dyspnea, and anxiety.
Hypotension and bronchospasm are deadly.
- Usually if elective surgery
DIC and acute kidney injury may occur
- Done 4-6 weeks prior to surgery
Hemoglobin rapidly releases and excreted Management:
by the kidneys (hemoglobinuria) - Intubation
- Fluid support
Management: Delayed hemolytic Extravascular hemolysis via RES when
- DQ immediately! reaction antibody levels has increased
- Notify physician
Allergic reaction Manifests as urticaria and flushing Manifests as fever, anemia, and jaundice
Iron overload Usually in long term
Management:
- Antihistamine (diphenhydramine)
Circulatory Happens when infusion is too fast or Pharmacologic alternatives to blood transfusion
overload circulatory system not normally functioning 1. Growth factors
2. Erythropoietin
Manifests as dyspnea, tachycardia, and high
3. Granulocyte colony-stimulating factor (filgrastim)
BP
4. Granulocyte-macrophage colony-stimulating factor
Management: (sargramostim)
- Diuretics 5. Thrombopoietin
- Patient placed upright with feet
dangling
- DQ or slow infusion depending on GUIDE QUESTIONS
severity
Transfusion-related HLA or HNA reacts to recipient’s leukocytes 1. First three priority actions in patients having transfusion
acute lung injury or WBCs of donor reacts to antibodies of reactions
the recipient leading to alveolar edema and 2. Vaccinations forbidden in patients who had a recent blood
sequestration of WBCs into alveolar transfusion
capillaries

Onset: usually after 6 hours, often within 2


hours

Manifests as DOB, hypoxia, hypotension,


fever, pulmonary edema
- Fatigue
- General malaise
- Pallor
- Tachycardia on exertion

Assessment and Diagnostics

Section IV: Diseases and Disorders General: Hgb, Hct, Rct, MCV, RDW

Non-Malignant Disorders IDA: serum iron, TIBC, ferritin

Chapter I: Anemias BM defect: BMA

Introduction Bleeding: endoscopy

- Lower than normal Hgb concentration


- RBC is low
Medical Management
Pathophysiology
Nursing Management
1. Hypo-proliferation: low production
Assessment:
- Bone marrow defect
- Materials are low (B9, B12, iron, EPO) 1. Review medications
2. Hemolytic: high destruction 2. Alcohol intake
- RBC defect: G6PD & SCD, spherocytosis 3. Dietary intake
- Plasma antibodies: immune hemolytic anemias 4. Fecalysis
- Mechanical destruction: heart valve defect 5. Neurologic assessment
3. Bleeding: high loss
Diagnosis and Intervention
Note: normal reticulocytes means bone marrow is responding
1. Managing fatigue
properly to hypoxia
- Balance between activity and rest
General symptoms: - Provide assistance
2. Maintaining adequate nutrition
*rapid loss = more severe symptoms
- No alcohol
- Weakness - Oral supplements (B9, B12, iron)
- Food preferences - About 6-12 months
3. Managing activity intolerance - On an empty stomach if tolerated
- IV fluids (1hr before or 2hrs after meals)
- Oxygenation Nx
4. Adherence to therapy - Organ meats, leafy greens, orange
- Iron supplements may cause GI distress juice, raisins, molasses
- Constipation and cramping may
- Abrupt steroid withdrawal effects
occur. Take supplement with food.
5. Monitoring potential complications
- Gradual iron intake (1 – 2 – 3)
- Heart failure
- High fiber diet
- Neurologic dysfunctions - Stools will be dark
Hypo-proliferative Anemias - Liquid oral iron may stain teeth.
Use straw.
Iron Deficiency Pathophysiology
Anemia - Iron store is inadequate Anemias in Renal Due to low EPO production
- Bleeding: ulcers, menstruation Disease
- Malabsorption: Crohn’s, gastritis, Anemia of Poor GIT absorption, RBC macrophage
celiac disease Inflammation sequestration
- Low consumption: vegetarians Normochromic, normocytic
SS Iron supplement not beneficial
- Smooth, red tongue Aplastic Anemia Pathophysiology
- Pica - Etiology: Viral infection,
- Restless leg syndrome autoimmune (T-cells),
- Angular cheilosis radiation/chemotherapy, drugs
- Brittle nails (chloramphenicol, sulfonamides),
Ax and Dx chemicals (benzene)
- BMA is definitive but not common - Bone marrow stem cells are
- Ferritin levels is most precise (iron damaged and destroyed (replaced
storage) by fat)
- Low serum iron, MCV, Hct, and RBC - Pancytopenia
- TIBC is high (measures transferrin) SS
Medx - Anemia: fatigue, pallor
- Ferrous sulfate (more iron) - Thrombocytopenia: bruising
- Ferrous gluconate (less iron) - Neutropenia: infections
- Ferrous fumarate
Ax and Dx pregnancy)
- BMA: aplasia (little to no stem cells) - Large RBCs causing hemolysis
- CBC: pancytopenia, fatty
- Reticulocytes: low B12 Deficiency
Medx - Diet is deficient (less common)
- HSCT (TOC) - Chronic gastritis or use of antacids
- Immunosuppression: - Little to no intrinsic factor
antithymocyte globulin: may cause (pernicious anemia)
fever and chills - B12 cannot bind to ileal enterocyte
- Cyclosporine - Impaired DNA synthesis and myelin
- Premedicate with corticosteroids sheath formation
- Anti infectives
- PRBCs and platelet SS
Nx - Beefy, red tongue
- Monitor signs of infection and - B12: Mild diarrhea, vitiligo, gray
bleeding hair
- Monitor side-effects of medications - Neurologic symptoms w/B12:
- Cyclosporine: monitor renal and paresthesia, loss of balance and
liver function proprioception
Megaloblastic Features: Ax and Dx
Anemias - Pancytopenia - B9/B12 serum level
- Poikilocytosis - Schilling test: how well B12 is
- Hemolysis absorbed using radioactive solution
Pathophysiology - Intrinsic factor antibody test
- Hormones needed for RBC Medx
production: T3, T4, EPO - Folate 1mg daily (IM or oral)
- Nutrients: iron, B9, B12 - B12 IM monthly
- 4-8 weeks before normal RBC count
Folic Acid Deficiency Nx
- Folate is used to form DNA - Promote safety: stability during
nucleotides ambulation, avoid extreme heat or
- Deficient B9 leads to impaired DNA cold
synthesis (diet low in green leafy - Soft and bland foods
veggies and organ meat or in
Hemolytic Anemias Nx
- RBC has short lifespan Hereditary Pathophysiology
- Increased EPO Hemochromatosis
- High reticulocytes SS
- High bilirubin Ax and Dx
- Low haptoglobin Medx
Sickle Cell Disease Features Nx
- Autosomal recessive (HbS gene) Hear valve defect Fragmented RBCs
- 10-20 days lifespan
- Sickling process triggered by low
oxygen tension Chapter II: Polycythemia
Pathophysiology
Primary Secondary
- Beta gene mutation in Hgb
- Hgb strands make up the rigid
structure of the cell
- High risk for occlusion and ischemia Chapter III: Neutropenia
SS
- Hemolysis: jaundice, enlarged bone Pathophysiology
marrow (face and skull)
SS
Ax and Dx
Medx Medx
Nx
Thalassemia Nx
G6PD Deficiency Pathophysiology
Chapter IV: Lymphopenia
SS
Ax and Dx
Medx
Nx Chapter V: Bleeding Disorders
Hereditary
Pathophysiology
Spherocytosis
Immune Hemolytic Pathophysiology SS
Anemias SS
Ax and Dx Mx
Medx
Nx
Thrombocytopenia Pathophysiology Disorders Vitamin K deficiency
Heparin-induced Thrombocytopenia
SS Disseminated Pathophysiology
Ax and Dx Intravascular
Medx Coagulation SS
Nx Ax and Dx
Immune Pathophysiology Medx
Thrombocytopenic Nx
Purpura SS
Ax and Dx
Medx
Nx Chapter VI: Secondary Thrombocytosis
Platelet Defects Pathophysiology

SS
Ax and Dx
Medx Chapter VII: Thrombotic Disorders
Nx
Inherited Bleeding Hemophilia Pathophysiology
Disorders Pathophysiology
Medx
SS Nx
Ax and Dx
Medx Hyperhomocysteinemi Pathophysiology
Nx a SS
Ax and Dx
vWD Medx
Pathophysiology Nx
Antithrombin
SS Deficiency
Ax and Dx Protein C Deficiency Pathophysiology
Medx SS
Nx Ax and Dx
Acquired Bleeding Liver disease Medx
Nx - Induction: cytarabine +
Protein S Deficiency daunorubicin/idarubucin/mitoxantrone
Activated Protein C . Healthy cells also die. Hospitalized 4-6
resistance and Factor V weeks. ANC may be as low as zero!
Leiden Mutation - Consolidation: lower doses
Antiphospholipid - Hydroxyurea
antibody syndrome - Granulocyte growth factors (except
Malignancy with APL type)
Complications
- Bleeding and infection are the most
GUIDE QUESTIONS FOR NON-MALIGNANT CONDITIONS common cause of death
- Tumor lysis syndrome: electrolytes spill
3. Priority interventions for IDA? Additional labs beside CBC?
out (potassium, phosphate, uric)
4. Challenges in SCD patient wanting to get pregnant. History?
CML Features
Other members involved? - 15%nof leukemias
5. Risks for hemochromatosis? Best treatment options? - Average age upon diagnosis: 67
Malignant Hematologic Neoplasms - BCR-ABL gene (Philadelphia
chromosome)
Chapter VIII: Leukemias - Chromosome 22 switches with
chromosome 9
AML Features: Phases
- Most mortality - Chronic: no problem
- Most common - Accelerated: transformation to acute
SS form, symptoms start arising
- Neutropenic: fever, infection - Blast phase: presents like AML, WBCs
- Anemic: pallor, weakness crowd capillaries impeding blood flow
- Thrombocytopenic: bruises, bleeding to the brain or lungs
- Splenomegaly Mx
- Hepatomegaly - Tyrosine kinase inhibitors: imatinib
- Bone pain (hyperplasia) mesylate (periorbital edema), dasatinib
Ax and Dx (myelosuppressive, pleural effusion,
- CBC long QT), nilotinib (cardiotoxic)
- BMA: >20% blast cells (hallmark) - Cytochrome P450 pathway means lots
Mx of drug interactions
- Lowers potency of TKIs: corticosteroids, - Consider: tolerance (creatinine
antiseizures, antacids, St. John’s wort clearance, ADL)
- Increases potency of TKIs: azole - Monoclonal antibodies: rituximab,
antifungals, clarithromycin, grapefruit alemtuzumab (targets CD52 antigen)
juice - Chemo agents: fludarabine
ALL Features (myelosuppression),
- 75% from precursor B-cell cyclophosphamide, bendamustine,
- 25% from precursor T-cell chlorambucil)
- Median age at diagnosis: 15 - Vancyclovir as prophylactic antiviral
- Common in boys Nx
- High incidence of organ infiltration
(brain: nerve palsies, headache, Nursing Management of Acute Leukemia
vomiting)
Mx 1. Preventing and managing infection and bleeding
- Intrathecal chemotherapy for CNS 2. Improving nutritional intake
involvement - Oral care before and after eating
- Induction: dexamethasone (avascular - Analgesic before eating
necrosis), vinca alkaloids, anthracycline - Antiemetics
+ asparaginase (thrombosis) - Small, frequent feedings
- Soft, moderate temp food
CLL Features - Avoid uncooked foods
- Most common adult leukemia - Peel fruits
- Strongest familial predisposition 3. Easing pain and discomfort
- Most cells are mature
- Linen change
- Lymph nodes and spleen infiltration is
- Back rub
common
- Active listening (psychological)
- Can transform to lymphoma (Richter’s
transformation causing B-symptoms – - Acetaminophen
fever, night sweats, weight loss) - Sponge bath
SS 4. Managing fatigue and activity intolerance
Ax and Dx - Balance between rest and activity
- Immunophenotyping - Be careful when ambulating outside if neutropenic
- B2-microglobulin on surface - Stationary bike inside the room
Mx - Sleep is important
5. Maintaining fluid and electrolyte balance - Antithymocyte globulin + cyclosporine or corticosteroid
- Assess for signs of dehydration or fluid overload - Transfusions
- Bun, creatinine, basic metabolic panel - Epogen
6. Improving self-care - Myeloid growth factors
- Limitations are temporary - Recombinant thrombopoietin receptor agonists:
- Assist as needed romiplostim, eltrombopag
- Promote independence - Iron chelating therapy (deferasirox) taken in the evening
7. Managing anxiety and grief prior to dinner, increased gradually to decrease diarrhea and
- Support system abdominal cramping as side-effect. Not given in cirrhosis.
8. Encouraging spiritual well-being Usually increases creatinine levels.
- Pneumonia is common

Chapter IX: Myelodysplastic Syndromes


Chapter X: Myeloproliferative Neoplasms

Pathophysiology: RBCs, WBCs, platelets die inside the marrow


before being released in the blood stream or decreased lifespan in Polycythemia Vera Features
the blood stream. These are dysfunctional cells. - Hct: .60%
- Survival: 14-20 years
Features: old age, males, idiopathic, risks include exposure to - Median age of onset: 60
alkylating agents, chemotherapy, and radiotherapy Pathophysiology: hyper-viscosity
SS
SS: anemia, infections, bleeding, can progress to AML
1. Neurologic: headache, dizziness,
Ax and Dx: BMA and biopsy as definitive TIA, vision changes
2. Cardiovascular: angina, ruddy
Medx complexion, thrombophlebitis,
dyspnea, claudication,
- Hypomethylating agents: promotes tumor suppressor genes
hypertension
(azacytidine, decitabine) used if EPO-stimulants not working
3. Fatigue and night sweats
anymore as it may alter disease progression 4. High basophils = histamine =
- Curative: HSCT pruritus
- Lenalidomide: for chromosome 5q deletion type, and should 5. Erythromelalgia: red, painful
not be thrombocytopenic fingertips and toes
6. Thrombotic events as most Primary Pathophysiology: JAK2 or CALR mutation
common cause of death Myelofibrosis leading to increase in fibroblast growth
factor replacing HSC, leading to
Risk classification extramedullary hematopoiesis. Death
1. Low risk: <60 y/o w/o history of occurs as a result of pancytopenia or
thrombosis thrombotic events
2. High risk: >60 y/o or history of Medx: JAK 2 inhibitor (ruxolitinib), HSCT
thrombosis or both Nx

Medx
- Phlebotomy (mainstay): 500 mL
once or twice a week
- Aspirin
- Cytoreduction: hydroxyurea
- Jak2 inhibitor: ruxolitinib if cannot
tolerate hydroxyurea (causes
anemia and thrombocytopenia)
Nx
1. Fatigue: EPO, exercise, sleep
2. Pruritus: tepid bath, oatmeal-
based lotion
3. DVT: no cross legs, exercise, no
restrictive clothing
Essential Features
Thrombocythemia - High megakaryocytes/platelet
- Jak2 mutation more deadly than
CALR gene mutation
SS
- Almost same with polycythemia
vera
Ax and Dx
- Plt count >450 up to 2million
Medx
- Same with polycythemia
1. Nodular sclerosis: fibrotic nodes,
highly curable
2. Mixed cellularity: almost equal
amounts of normal lymphocytes
and RSC
3. Lymphocyte-rich: lymphocytes >
RSC
4. Lymphocyte-depleted:
lymphocytes<RSC
5. Nodular lymphocytes
predominant HL: few RSC but
lymphocytes appear like
“popcorn” (multi-folded nucleus)

SS
- Cervical lymph node
enlargement: painless, firm but
not hard
- B-symptoms (fever, night sweats,
weight less of 10%)
Chapter XI: Lymphoma - Due to tumor compression:
trachea (DOB), lungs
(cough/effusion), spleen
Hodgkin Lymphoma Features (splenomegaly, abdominal pain),
- High cure rate (90%) liver (jaundice), bone (pain)
- RF: viral infections (HIV, HHV8, - Pruritus
EBR), immunosuppressive Ax and Dx
therapy, family history - High ESR
- Hallmark: Reed-Sternberg cell - Mild anemia
(multinucleated, owl-like) - Excisional biopsy (+) RSC
- Unicentric: arising from single - CXR and CT for extent of
node adenopathy
- PET for staging
Types Medx
- Doxorubicin (Adriamycin) (cyclophosphamide,
- Bleomycin hydroxydaunorubicin, vincristine,
- Vinblastine prednisone) + MoAb (rituximab)
- Dacarbazine Complications of treatment
- If relapse: monoclonal Ab - Tumor lysis syndrome
(everolimus, brentuximab), - Reactivation of TB
checkpoint inhibitor (nivolumab, - Multifocal leukoencephalopathy
pembrolizumab) - Cardiovascular, fertility problems
- If more advance, radiation is - Secondary malignancies: AML,
added MDS
Nx
- Avoid: tobacco, alcohol, excessive
sunlight, carcinogens
Non-Hodgkin Features
Lymphoma - High morphological variability
- Highly invasive

SS
- Similar to Hodgkin but more
aggressive

Ax and Dx
- Flow cytometry: detects specific
antigen
- Fluorescence in situ hybridization
(FISH): detects RNA/DNA for
chromosomal abnormality
- Biopsy, X-ray, PET

Medx Chapter XII: Multiple Myeloma


- Asymptomatic: watch and wait
- Indolent: radiation therapy alone Pathophysiology
may suffice
- Aggressive: CHOP
Ax and Dx

Medx

Nx

GUIDE QUESTIONS FOR MALIGNANT CONDITIONS

1. Possible complications 1 week post-induction therapy for


AML
2. Key assessment and laboratories for multiple myeloma?
Comorbidities that could impact the treatment
3. Treatment options for B-cell lymphoma? Adverse effects?
EBP?

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