Hematology
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| Autosomal recessive, pain in extremities after exercise, priapism | Sickle cell disease
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| Thalassemia genetics | alpha: 2/2 gene deletion (2 genes on chromosome 16); beta: 2/2 point mutation (1 gene on chromosome 11)
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| Microcytosis out of proportion to the anemia = | thalassemia
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| Thalassemia: alpha globin chains | 1 chain = Hgb H dz; all 4 chains deleted: hydrops fetalis (stillbirth); silent carrier = 1 gene deleted (nml MCV). Hgb A (trait)
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| thalassemia tx | Avoid Fe; Hgb H: folate supp, poss splenectomy; beta: transfusions (deferoxamine vs hemosiderosis); allo BMT
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| Cooley anemia AKA: | beta thalassemia major
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| beta thalassemia major S/S | sx onset 4-6 mos old; growth probs, abnormal facies, fx's, osteopenia, bone deforms, HSM, jaundice
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| Splenectomy: PPx tx includes: | Pneumococcal vaccine
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| SCD complications | AVN of femoral head; cholelithiasis, splenomeg, Strep pneumo infxn, stroke, priapism, retinopathies, osteomyelitis
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| Due to spectrin protein in cytoskeleton | Hereditary spherocytosis
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| Triad: anemia, splenomegaly and jaundice | Hereditary spherocytosis
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| Episodic hemolytic anemia related to oxidative stress (hereditary) | G6PD def
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| 2 RBC conditions w/pigment gallstones | Hereditary spherocytosis, sickle cell
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| Degrees of thalassemia disease | Hydrops fetalis = all 4 genes deleted (fatal); Hgb-H dz = 3 deleted (MCV=60-70); A-thal trait = 2 genes (MCV=70-80); silent carrier = 1 gene deleted
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| Thalassemia: Hgb-H dz labs | Hgb elect = 10-40% Hgb H; retic is high
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| Beta thalassemia types | Major (Cooley's): both beta globin genes mutated, severe anemia, txn dept; intermedia: txn in stress; minor rarely need txn
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| Tx for Hgb H disease | Splenectomy? & folic acid
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| Tx for beta thalassemia major | Txn, splenectomy, Fe chelation; allogeneic BM trans to cure
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| Enzyme disorder: adequate Fe but can't get it into Hgb; Fe accumulates in ? | Sideroblastic anemia; in RBC mitochondria
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| Sideroblastic findings | High serum Fe, large spleen/liver; mod anemia, low MCV; BM sideroblasts
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| Tx: Sideroblastic anemia | Dept on cause (ETOH, Pb, leukemia, TB Rx); txn/chelation
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| Anemia due to spectrin protein in cytoskeleton | Hereditary spherocytosis
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| Triad: anemia, splenomegaly and jaundice | Hereditary spherocytosis
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| Pigment type gallstones; chronic leg ulcers | Hereditary spherocytosis
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| Hereditary spherocytosis findings | Mod micro anemia; reticulocytosis; high indirect bili; Neg coombs
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| Hereditary spherocytosis tx | Folic acid 1mg po qd; splenectomy for severe
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| G6PD def prevalence | >200M worldwide (usu AA men); X linked recessive; females rare
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| Stress triggers of G6PD def | Infxns; toxins/drugs (sulfa/antimalarial); fava beans
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| G6PD deficiency findings | N/N anemia. No splenomegaly or chronic hemolytic anemia; bite cells/Heinz bodies; Reticulocytosis and increased bili in crisis.
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| G6PD def tx | Splenectomy for severe; (txn rarely); avoid stressors (ASA,sulfa, antimal, fava)
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| Autosomal recessive disorder in which abnormal Hgb leads to chronic hemolysis | Sickle cell disorders
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| In sickle cell dz, rate of sickling depends on: | Conc of Hgb S, dehydration, presence of other Hgb (F), hypoxemia & acidosis
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| Sickle cell prevalence | 8% of AA: Hgb S gene; 1/400 AA have SSA; onset in 1st yr (Hgb F falls); 40-50 yr life exp
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| S/S = Pallor, jaundice, splenomegaly, leg ulcers, pigment gallstones, priapism | Sickle cell
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| Sickle cell: vascular occlusion leading to infarction | Pulmonary (acute chest syndrome); Retinal (blindness?); Renal (to renal failure ); Stroke, even in kids; Splenic infarcts; aseptic necrosis of femoral head
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| Sickle cell tx | Folic acid 1 mg po qd; hydrate, low alt, vax (flu/pneumo/mening)
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| Sickle Cell A long-term issues | Splenectomy: infxn probs; narco abuse; Hydroxurea to increase Hgb F
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| Total absence of alpha-globin genes = | hydrops fetalis (Hgb Bart's: tetramer of 4 gamma globulin chains). Results in stillbirth
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| SCD pathology | Hgb S occurs when valine substitutes for glutamic acid on beta-chain. Decreased Hgb S solubility in hypoxic tissues -> sickling
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| Most severe form of thalassemia | Beta thal major (minor is like iron def anemia). FTT, Mediterranean.
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