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Hematology

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Answer
Microcytic anemia: DDx   TICS: thalassemia, iron deficiency, chronic inflammation, sideroblastic  
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Macrocytic anemia suggests:   folate or B12 deficiency  
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Anemia: RBC production problem (vs destruction problem) distinguished by:   Retic count; hypoproliferative = retic low (<2); hyper = retic high (>3)  
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Causes of Fe deficiency anemia   Poor diet/intake; decreased absorption (atrophic gastritis, GI surg, sprue, achlorhydria); increased loss (GI bleed, menorrhagia, neoplasm); increased requirements (PG, lactation)  
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Anemia of chronic dz: lab pattern   Likely microcytic. Low Fe, low ferritin, high TIBC. Low erythropoietin  
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Glossitis, peripheral neuropathy (stocking-glove paresthesias); MCV 110-140; low retic, hyperseg PMNs, high methylmalonic acid =   B12 def  
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Triad: anemia, splenomegaly and jaundice =   Hereditary spherocytosis  
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Fe deficiency anemia tx   ferrous sulfate 325mg tid  
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pernicious anemia tx   lifelong B12 supplementation  
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Hx HTN, nephrotic syndrome, CRF or Renal insufficiency; do CBC:   Anemia  
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PICA, Pregnant & fatigue, Menstruation; cheilosis:   Fe Deficiency Anemia  
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Anemia after colectomy, partial gastrectomy; glossitis, decreased vibratory sensation =   B12 or Pernicious anemia  
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G6PD + Quinidine, Nitrofurantoin, Sulfa, fava beans =>   Hemolytic anemia  
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Fatigue, weakness, low fever, purpura, pallor, gingival bleeding; low retic; no HSM =   Aplastic anemia  
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folate def is ______ anemia; erythropoeisis is due to lack of:   megaloblastic anemia; lack of methylation  
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sideroblastic anemia tx: pts do not respond to:   EPO; tx underlying cause, poss txn/ chelation  
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sideroblastic anemia causes   myelodysplasia, chronic EtOH, Pb tox  
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N/N anemia pathophysiology   hepcidin upregulation in response to inflammatory mediators  
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N/N anemia etiology   organ fail d/t systemic dz (renal, endo, thy, liver), or impaired marrow fn (2/2 chronic infxn, aplastic anemia, PRCA)  
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macro anemia 2/2 folate def: S/S   glossitis, no neuro sx  
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macrocytic anemia 2/2 B12 def S/S   glossitis, pale icterus, GI sxs; neuro sxs (posn, vib sense, stocking-glove paresthesia, ataxia)  
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B12 def causes   No. 1 pernicious anemia; veganism, blind loop syndrome, gastric surg, panc insuff, Crohn dz  
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Most common cause of anemia worldwide   Fe def anemia  
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HYPOproliferative anemia due to:   Marrow damage, Fe def, dec'd stimulation  
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HYPERproliferative anemia due to:   BM failure to compensate for blood loss or RBC destruction  
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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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Causes of B12 def   Diet (vegans); malabsorption: inadequate intrinsic factor prodn (70%), terminal ileum dz (sprue, Crohns), tapeworm, drugs  
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B12 def vs folic acid def   B12: meat/dairy; to 5000 mcg in liver (3 yrs); Folic acid: fruit/veg; 5-20 mg stored (few mos)  
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Acquired stem cell disorder making RBC membrane prone to lysis by complement   PNH  
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In PNH, there is increased risk of:   leukemia/myelofibrosis  
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Acquired hemolytic anemia due to IgM autoantibody   Cold agglutinin dz  
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Evans syndrome   (immune thrombocytopenia) AIHA  
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Anemia: morphology relative to MCV   <80 = microcytic; 80-100 = normo; >100 = macro  
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Anemia: RBC production problem (vs destruction prob) distinguished by:   Retic count; hypoproliferative = retic low (<2); hyper = retic high (>3)  
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Hypoproliferative anemia due to:   Marrow damage, Fe deficiency, decreased stimulation  
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Hyperproliferative anemia due to:   BM failure to compensate for blood loss or RBC destruction  
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Most common cause of anemia worldwide   Fe deficiency anemia  
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Macrocytic anemias   Folic acid def; B12 def  
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Tx B12 def   Replacement tx: IV B12; frontload dose  
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B12 def vs folic acid def   B12: meat/dairy; to 5000 mcg in liver (3 yrs); Folic acid: fruit/veg; 5-20 mg stored (few mos)  
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Causes folic acid def   Inadeq intake (ETOH, teens, elder); inc’d req: PG, malignancy, anemias, hemodialysis; Malabsorption [sprue, drugs (phenytoin, barbituates)]; Impaired metabolism (ETOH, methotrexate)  
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Folic acid def findings   Malnourished; glossitis, cheilitis; NO neuro S/S; RBC folate <150 ng/mL  
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PRCA is contracted:   Acquired or idiopathic  
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Autoimmune dx mediated by T lymphocytes or (rarely) IgG antibody against erythroid precursors   PRCA (pure red cell aplasia)  
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Poss causes include thymoma, solid tumors, SLE, RA, HIV/hep, phenytoin   PRCA  
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PRCA findings   Normal lab except low retic; severe anemia S/S  
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PRCA Tx   Stop all meds; poss thymoma resection; IV Ig  
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Due to BM failure (injury or suppression); pancytopenia   Aplastic anemia  
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Causes of aplastic anemia   Idiopathic (50-65% ; likely autoimmune); Phenytoin, sulfonamides; chemotherapy, radiotherapy; benzene, solvents, insecticides; hep, HIV, EBV; PG, SLE, GVHD; Hereditary (rare): Fanconi’s anemia  
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Aplastic anemia findings   NO hepatosplenomegaly; pancytopenia (purpura, pallor, petechiae); low retic; hypocellular BM  
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Aplastic anemia: diff dx from: 1-MDS; 2-hairy cell; 3-normo   1=abn cells; 2=splenomegaly & abn lymph cells; 3=SLE, hypersplenism, dissem'd infxn  
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Aplastic anemia tx   Family HLA typing; supportive tx (txn/infxn); immunosuppn; GF; BM txplant  
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Aplastic anemia prognosis   Untx'd = fatal; HLA matched txplant: 60-90% cure; ATG tx: partial remission 60-80% (1/3 relapse & 20-50% MDS)  
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Hemolytic anemias: intrinsic vs extrinsic   Intrinsic = prob w/membrane, z defects, hgb (usu hereditary); Extrinstic = autoimmune, drugs or mechanical trauma (often acquired)  
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Hemolytic anemia lab findings   Low haptoglobin; high LDH, indirect bili (not >4-5mg/dL unless underlying liver dz), retic; hemoglobinuria; stable or falling hgb  
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Workup to distinguish etiology of Hemolysis   Clinical & FH; Direct Coombs; Peripheral blood smear; morpho (Heinz bodies, sickle cells, parasites); Hgb electrophoresis  
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Acquired stem cell disorder making RBC membrane prone to lysis by complement   PNH  
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PNH findings   Episodic hemolytic anemia; Venous thrombosis; Deficient hematopoiesis -> pancytopenia; reddish-brown urine; Hemoglobinuria, esp. first morning urine  
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Episodic hemolytic anemia; Venous thrombosis; Deficient hematopoiesis -> pancytopenia ... indicate:   PNH  
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In PNH, increased risk of:   leukemia/myelofibrosis  
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PNH tx   Tx Fe def; prednisone for hemolysis; BM txp if severe  
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Acquired disorder which IgG autoantibody formed that binds to RBC membrane   AIHA (autoimmune hemo anemia)  
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AIHA: ___% are idiopathic; seen in ___   50% ; lupus, NHL, CLL  
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AIHA findings   Pos coombs test; severe anemia (6-10); high retic & ind bili; spherocytes & NRBC; Evans syndrome (immune thrombocytopenia)  
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AIHA tx   Prednisone 1 mg/kg/d; splenectomy if fail; Rituximab, Danazol, IVIG if fail; avoid txn  
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Acquired hemolytic anemia due to IgM autoantibody   Cold agglutinin dz  
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Cold agglutinin dz causes   Idiopathic or neoplasm or post-infxn (Monoclonal gammopathy, mono, mycoplasma)  
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Cold agglutinin dz findings   Mottled/numb fingers/toes; anemia is rarely severe; mild reticulocytosis; spherocytes; Pos Coombs for complement; Pos cold agglutinin test; Chilled blood will look clumped on slide  
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Cold agglutinin dz tx   Symptomatic, avoid cold; poss Rituximab or immunosupp or plasmapheresis; no splenectomy/steroid  
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Evans syndrome   (immune thrombocytopenia) AIHA  
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Neutropenia findings   Stomatitis; fever; poss septicemia, pneumonia and cellulitis  
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Neutropenia tx   DC potl causative agents; tx infxns; poss GF (G-CSF) for severe  
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Anemia: no hepatosplenomegaly   aplastic anemia  
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Anemia without neuro Sx/Sx suggest:   Folic acid def  
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Aplastic anemia sx/sx do not include:   hepatosplenomegaly  
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Dark blue-gray line along gingival border may be sx of:   lead poisoning  
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most common anemia of hospitalized patients =   anemia of chronic disease  
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Anemia of chronic disease etiologies   Chronic inflammatory conditions: infection, IBD, RA, malignancy. CKD  
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Macrocytic anemia etiologies (PALER)   Pernicious anemia. Lung (COPD). EtOH (AST > ALT 2x, increased GGT). Rx (antiseizure meds, methotrexate)  
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Elevated retic count may be due to:   Hemolytic anemia; acute blood loss; response to therapy (within 5-7days)  
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Low or Normal retic count may be due to:   All other forms of anemia (other than hemolytic or acute blood loss)  
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