Hematology Flashcards
Polycythemia Vera defining symptoms and mutation
1) Generalized pruritis
2) Burning pain on hands/arm with blueish red discoloration (erythromelalgia)
3) JAK2 mutations (EPO receptor)
Can also have leukocytosis and thrombocytosis
Can cause gout (increased cell turnover) and myelofibrosis
Tumor lysis syndrome electrolyte changes
Hyperkalemia
Hyperphosphotemia
Hyperuricemia
HYPOcalcemia
Paroxysmal nocturnal hemoglobinuria
Phosphatidyl-inositol glycan A (PIGA) mutation that prevents GPI anchor formation
No decay accelerating factor (DAF or CD55) to prevent C3 convertase formation
Hemolysis
Cytopenia
Venous thrombosis
Hereditary spherocytosis tests
Osmotic fragility test
Eosin-5-maleimide binding test
MCHC increased due to membrane loss
Hemoglobin A2
a2d2
elevated in beta-thalassemia
Hemoglobin Barts
y4
Hemoglobin H
b4
Folate (B9) vs Cobalamin (B12)
Both required for conversion of homocysteine to methionine (elevated homocysteine)
Only B12 required for methylmalonyl-CoA to succinyl-CoA (elevated MMA)
Type I vs type II HIT timing
Type I: 1-2 days (rarely below 100K)
Type II: 5-10 days (50% reduction)
Common variable immunodeficiency
Mostly low igg
Don’t respond to immunization
Increased risk of autoimmune
Increased risk of nonhodgkins
hereditary thrombophilias
1) factor V leiden (resistant to activated protein C)
2) prothrombin
3) antithrombin
4) protein c/s deficiency
total body stores of B12 and folate
B12 - 4 years
Folate - 4 months
TTP vs HUS
Both cause MAHA and thrombocytopenia
HUS is more renal and usually has not AMS or fever
pyruvate kinase deficiency
chronic hemolysis
pigmented gallstone
skin ulcers
HSM
CLL s/sx
LAD HSM Severe lymphocytosis w/ smudge cells Diagnose with Flow (LNBx useful for hodgkins/nonhodgkins)
Can get frequent infections
Autoimmune hemolytic anemia
Richter transformation
