Sickle/ Thallessemia

Question 1

• SCD: Heredity? From what?
• Can also occur with? (2)
• CBC has? (7)
• HbSS RBC Size? HbSB+/0? HbSC? Retic counts? Level of severity?
• Deoxygenated leads to? After multiple cycles?
• Signs: Retic? Pt? WBC? RDW? Billi? AAT?
• Big concern? What can cause this? RBCs survive? Even when not sickled?
• Increased occlusion risk where? Occurs with (4)

Answer 1

• AR; both B globins (glu-val)
• HbC; thallassemia
• Sickle cells, schistocytes, polychomsia, anisocylsis (size), poikilocytosis (shape), Howell- Jolly (no spleen); target cells with HbSC
• Normal; Small; Normal; All high; HbSS>HbSBo.HbSC> HbSB+
• Sickle; permanent
• Up for all
• Aplastic crises; Parovirus B19; 20 days; more sticky
• Microvasculature; hypoxia; dehydration, infection, inflammation

Question 2

• HbSS treatment? (5)
• HbA? HbA2? HbF? HbH? HbBarts?
• Alpha thal? 4 types? MCV?
• Beta Thal? B+? B intermedia? Cooleys? MCV?
• Retic? RDW? MCV? Billi? AST? LDH?
• Smear? (4)
• Clinical manifestations? (5)
• Treatment? (3)

Answer 2

• Folic acid, penicillin, bone marrow transplant, hydroxyurea (Incr. Fetal Hb), Transfusion
• A2B2; A2D2; A2Y2; B4; Y4
• Absense of 1-4 alpha genes; silent; 2 gene deletion; HbH disease; Hydrops Fatalis
• Underproduction of beta globin; 1 normal/1 abnormal; 2 mildly abnormal; 2 abnormal/absent; low
• up; normal; low; high; high; high
• Microcytosis; target; sombraro
• Splenomegaly; expanded bone marrow; incr. Fe absorp; pulm. hypertension; endicrinopathies
• Transfusion; hydroxyurea; bone marrow transplant