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ANEMIA 5-2• Special clinical manifestations (in severe cases): chipmunk facies, pathologic fractures,hepatosplenomegaly (due to extramedullary hematopoiesis), high-output CHF,bilirubin gallstones, iron overload syndromes (from chronic transfusions)• Diagnosis: MCV 70, normal Fe, MCV/RBC count 13, c retics, basophilicstippling; Hb electrophoresis: c HbA 2 ( 2 2) in -thal; normal pattern in -thal trait• Treatment: folate; transfusions deferoxamine, deferasirox (oral iron chelator);splenectomy if 50% c in transfusions; consider allogeneic HSCT in children w/ severe-thal majorAnemia of chronic inflammation (see below)Sideroblastic anemia• Defective heme biosynthesis within RBC precursors• Etiologies: hereditary/X-linked (ALAS2 mutations), idiopathic (MDS-RARS),reversible (alcohol, lead, isoniazid, chloramphenicol, copper deficiency, hypothermia)• Special clinical manifestations: hepatosplenomegaly, iron overload syndromes• Dx: review social, work, & TB hx; can be microcytic, normocytic, or macrocytic;variable pop of hypochromic RBCs; c Fe, nl TIBC, c ferritin, basophilic stippling,RBC Pappenheimer bodies (Fe-containing inclusions), ring sideroblasts(w/ iron-laden mitochondria) in BM• Treatment: treat reversible causes; trial of pyridoxine, supportive transfusions forsevere anemia; high-dose pyridoxine for some hereditary casesFigure 5-2 Approach to microcytic anemias↓ Fe, ↑ TIBC↓ ferritinFe/TIBC 13↓ marrow Fenormal iron studiesMCV/RBC 18%↑ Fe, nl TIBC↑ ferritinbasophilic stipplingring sideroblasts in BMIron deficiencyanemiaThalassemiaAnemia ofchronic inflammationSideroblasticanemiaPancytopenia (see below)NORMOCYTIC ANEMIASAnemia of chronic inflammation (ACI; NEJM 2005;352:1011; 2009;361:1904)• T RBC production due to impaired iron utilization and functional iron deficiencyfrom c hepcidin; cytokines (IL-6,TNF-) cause T epo responsiveness/production• Etiologies: autoimmune disorders, chronic infection, inflammation, HIV, malignancy• Dx: T Fe, T TIBC (usually normal or low transferrin sat), c ferritin; usuallynormochromic, normocytic (70% of cases) but can be microcytic if prolonged.• Coexisting iron deficiency common. Dx clues include T serum ferritin levels, absenceof iron staining on BM bx, response to a trial of oral iron, and/or c solubletransferrin receptor/ferritin index (Blood 1997;89:1052).• Treatment: treat underlying disease erythropoietin (? if Epo 500 mU/mL); forcancer- or chemo-related ACI, use epo if Hb 10 g/dL. Iron if ferritin 100 orFe/TIBC 20%.Anemias of chronic disorders• Anemia of chronic inflammation (see above)• Anemia of chronic kidney disease: T epo; may see burr cells; treat w/ epo (see“Chronic Kidney Disease”)• Endocrine deficiencies: hypometabolism and T O 2 demand with thyroid, pituitary, adrenal,or parathyroid disease STepo; can be normocytic or macrocyticSideroblastic anemia (see above)Pure red cell aplasia• Destructive antibodies or lymphocytes S ineffective erythropoiesis• Associated with thymoma, CLL, and parvovirus infection• Diagnostic studies: lack of erythroid precursors on BM bx, other lines normal• Treatment: thymectomy if thymus enlarged; IVIg if parvovirus infection;immunosuppression if CLL or idiopathic; supportive care with PRBC transfusions;? erythropoietin receptor agonist if due to antierythropoietin Ab (NEJM 2009;361:1848)