HEMATOLOGY NOTES

FOR MEDICAL STUDENTS

byJ.R. Phillip MD, PhD

LULU EDITION

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PUBLISHED BY:J.R. Phillip MD, PhD on Lulu

Hematology NotesFor Medical Students

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

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RED BLOOD CELL DISORDERS

BONE MARROW ABNORMALITIES

Aplastic AnemiaCauses: 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) → ↓cellsTx: Mild → supportiveSevere → 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 apoptosisMegaloblastic anemia without hypersegmented neutrophils (in Vit B12 and folic acid deficiency, megaloblastic anemia with hypersegmented neutrophils)

Polycythemia Rubra VeraRemember that it's polycythemia, not polyerythremia; therefore, all three cell lines are increased, but RBC more prominentlyHct>54% males, >51% females, ↑ RBC mass (to rule out relative polycythemia), ↓EPO, ↑WBC, ↑platelets,Headache, dizziness, blurring of vision, pruritus after warm bathTx: Phlebotomy to keep Hct<45

Neonatal PolycythemiaHematocrit >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 C3More complement binds to RBC & intravascular hemolysis occursLoss of anchor for DAF (Decay Accelerating Factor)Dark brown/pink urine in the morningPancytopeniaMajor venous (mesenteric, hepatic vein) thrombosisDx: 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 SyndromesUsually > 60 yoDysplastic bone marrowBone marrow: hypercellular, abnormal cellsNeutrophils with bilobed nucleus (Pelger-Huet cells)Pancytopenia, anemia, infections, bleeding, evolution to acute leukemiaPluripotent hematopoietic stem cell disordersCauses: idiopathic, radiation, cytotoxicsTx: supportive, may be given GM-CSF

MyelofibrosisFibrotic bone marrowBx (marrow) → ↑collagen/reticulinAnemia, peripheral blood → ‘Teardrop RBC’Clinical: constitutional, marrow failure<10% convert to acute leukemia, esp. if alkylators used

MyelophthisisBone marrow replacementTumors, infections, granulomas, storage diseases (e.g. Gaucher’s)Anemia, peripheral blood → ‘Teardrop RBC’

GENETIC DISORDERS

HemochromatosisHFE gene mutationDx: ↑ ferritin, ↑ transferring saturation, gene studies (confirmatory)

Accumulation of iron in:Heart → CHFLiver → cirrhosisPancreas → diabetesTestis → impotenceSkin → pigmentationTx: phlebotomy, alt. DeferoxamineComplications: ↑ risk of hepatocellular carcinoma

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