HEMATOLOGY
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
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)
ABO Typing
Rhesus Factor
Crossmatching
- Blood of donor is mixed with the blood of the recipient to
test for agglutination
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
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
Section IV: Diseases and Disorders General: Hgb, Hct, Rct, MCV, RDW
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
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
Nx