HEMATOLOGY NOTES

FOR MEDICAL STUDENTS

by

J.R. Phillip MD, PhD

LULU EDITION

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PUBLISHED BY:

J.R. Phillip MD, PhD on Lulu

Hematology Notes

For Medical Students

Copyright © 2015 by J.R. Phillip MD, PhD

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RED BLOOD CELL DISORDERS

BONE MARROW ABNORMALITIES

Aplastic Anemia

Causes: Fanconi’s syndrome, chemicals (Benzene), medications (Chloramphenicol, gold), radiation, idiopathic, chemotherapy, malignancy (especially leukemias), infections (parvovirus)

Pancytopenia (normochromic, normocytic anemia)

Bone marrow biopsy (most specific test) → ↓cells

Tx: Mild → supportive

Severe → Allogenic bone marrow transplant if < 50 yrs old & HLA-matched sibling available), Immunosuppression (Antithymocyte globulin, Cyclosporine, Prednisone) if >50 yrs old or HLA-matched sibling not available

Blackfan-Diamond Syndrome (Congenital Hypoplastic Anemia)

Intrinsic defect of erythroid progenitor cells which results in increased apoptosis

Megaloblastic anemia without hypersegmented neutrophils (in Vit B12 and folic acid deficiency, megaloblastic anemia with hypersegmented neutrophils)

Polycythemia Rubra Vera

Remember that it's polycythemia, not polyerythremia; therefore, all three cell lines are increased, but RBC more prominently

Hct>54% males, >51% females, ↑ RBC mass (to rule out relative polycythemia), ↓EPO, ↑WBC, ↑platelets,

Headache, dizziness, blurring of vision, pruritus after warm bath

Tx: Phlebotomy to keep Hct<45

Neonatal Polycythemia

Hematocrit >65%

If increase on hematocrit on heel prick sample → Re-check sample from peripheral blood (hematocrit 5-15% lower than heel prick)

If symptomatic (hypoglycemia, hyperbilirrubinemia, cardiac or respiratory compromise) → hydration and partial exchange transfusion

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Acquired clonal stem cell disorder

RBC membrane sensitive to lysis by serum complement C3

More complement binds to RBC & intravascular hemolysis occurs

Loss of anchor for DAF (Decay Accelerating Factor)

Dark brown/pink urine in the morning

Pancytopenia

Major venous (mesenteric, hepatic vein) thrombosis

Dx: Screening → sucrose lysis test (RBC lyse when incubated with sucrose), Ham test (RBC lyse when incubated with acidified serum), confirmation → flow cytometry (no CD59)

Tx: Corticosteroids (Prednisone), androgen (Danazol or Danocrine), anticoagulation for thrombosis, BMT

Myelodysplastic Syndromes

Usually > 60 yo

Dysplastic bone marrow

Bone marrow: hypercellular, abnormal cells

Neutrophils with bilobed nucleus (Pelger-Huet cells)

Pancytopenia, anemia, infections, bleeding, evolution to acute leukemia

Pluripotent hematopoietic stem cell disorders

Causes: idiopathic, radiation, cytotoxics

Tx: supportive, may be given GM-CSF

Myelofibrosis

Fibrotic bone marrow

Bx (marrow) → ↑collagen/reticulin

Anemia, peripheral blood → ‘Teardrop RBC’

Clinical: constitutional, marrow failure

<10% convert to acute leukemia, esp. if alkylators used

Myelophthisis

Bone marrow replacement

Tumors, infections, granulomas, storage diseases (e.g. Gaucher’s)

Anemia, peripheral blood → ‘Teardrop RBC’

GENETIC DISORDERS

Hemochromatosis

HFE gene mutation

Dx: ↑ ferritin, ↑ transferring saturation, gene studies (confirmatory)

Accumulation of iron in:

Heart → CHF

Liver → cirrhosis

Pancreas → diabetes

Testis → impotence

Skin → pigmentation

Tx: phlebotomy, alt. Deferoxamine

Complications: ↑ risk of hepatocellular carcinoma

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