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Heme/Onc SVL
FA Heme Onc Chapter
Question | Answer |
---|---|
Iron Deficiency Anemia | Reduced iron due to chronic bleeding, malnutrition or absorption disorders, or increased demand. Leads to reduced heme synthesis. May manifest as Plummer Vinson Syndrome |
Plummer Vinson Syndrome | Esophageal webs, glossitis, Fe Deficiency anemia |
Alpha Thalassemia | alpha globin gene mutations leads to reduced alpha globin synthesis. Deletion of four genes is incompatible with life, Hb Barts and hydrops fetalis. Deletion of three genes, HbH. Deletion of 1-2 genes does not lead to anemia. |
Beta Thalassemia Minor | B thal minor: underproduced beta chain. Usually asymptomatic. Increased HbA2 (greater than 3.5%) |
Beta Thalassemia Major | Cooleys Anemia. Beta chain is absent, blood transfusion is required (secondary hemochromatosis). Crew cut skull, marrow expansion, chipmunk facies, increase HbF |
Lead Poisoning | Inhibits ferrochelatase and ALA Dehydratase. Reduced heme synthesis. Basophilc stippling due to reduced RNA degradation. |
Sideroblastic Anemia | Hereditary: X linked defect in ALA synthase gene. Reversible etiology: alcohol, lead. Increased iron, normal TIBC, increased ferritin. See ringed sideroblasts |
Treatment of Sideroblastic Anemia | B6 |
Folate Deficiency | Megaloblastic anemia, hypersegmented neutrophils, glossitis, reduced folate, increased homocysteine, normal MMA. Caused by malnutrition, malabsorption, phenytoin, methotrexate, TMP, pregnancy, hemolytic anemia, giardia, celiac sprue. |
B12 Def | Pernicious anemia, |