Mildly elevated bilirubin, normal liver enzy mes are seen in-
Ans. is 'd' i.e., AH of above o Mildly elevated bilirubin especially indirect and normal liver enzyme seen in hemolytic anemia,o In above question all causes hemolytic anemiao In severe hemolysis or hemolysis which cause liver damage may derange LFT.o Other causes of hemolysisHemolytic Anemias and Their TreatmentDIAGNOSISDEFECTL ABORATORY TESTSTREATMENTCELLULAR DEFECT'SMembrane Defects HereditaryspherocytosisCytoskeletal protein defectsSpherocytes on blood filmIfHb> lOg'dL and reticulocyte count < 10%-none Frequently involve vertical interactions of spectrin ankyrin, protein 3Negative Coombs test eliminates immune hemolysisIf severe anemia, poor growth, aplastic crises, and age <2 yr- transfusion Increased incubated osmotic fragility.'Folic acid, 1 mg qd Abnormal cytoskeletal protein analysis HereditaryelliptocytosisCytoskeletal protein defectsElliptocytes on blood filmMild types-no- treatment Frequently involveRBCs mildly heat-sensitiveChronic hemolysis-horizontal interactions of spectrin, protein 4.1, and glycophorin cAbnormal cytoskeletal protein analysistransfusion and splenectomy as recommended for spherocytosis (see above) Folic acid, 1 mgqdHereditarypyropoikilo-cytosisCytoskeletal protein defectssize and shape on bloodExtreme variation in RBC splenectomy as filmTransfusion andrecommended for spherocytosis (see above) Homozygous or double heterozygous abnormality in horizontal interactions of a spectrinThermal sensitivity7 fragmentation at45degC for 15 minFolic acid, 1 mg qdHereditarystomatocytosisCytoskeletal protein defectsStomatocytes on blood filmSplenectomy should be avoided (see text) Decreased protein 7.2b (1 subset)Abnormal RBC cation and water content Folic acid, 1 mgqdParoxysmal nocturnal hemoglobinuriaPrimary' acquired marrow disorderDecreased WBC CD55 and CD59 or decreasedFolic acid, l mg qd RBCs unusually sensitive to compl ement-medi ated lysisRBC CD59 by flow cytometryMild cvtopenias-no treatment Ham's test, sucrosis lysis testMarrow aspirate and biopsy to assess cellularityChronic hemolysis and other cytopenias- prednisone, 60 mg qd initially, then taper if possible; maintenance therapy 15-40 mg qod Decreased decay- accelerating factorIron for secondary iron deficiency Androgen s- fluoxymesterone Anticoagulation Marrow transplant for pancytopeniaEnzyme Deficiencies Pyruvate kinase deficiencyDecreased or abnormal enzymePyruvate kinase assay-decreased or rarely high Km variantIf severe anemia with symptoms, poor growth and age <2 yr-trans fusion Splenectomy > age 6 yr, but earlier if necessaryFolic acid, 1 mg qdG6PD deficiencyA- type:age-labile enzymeMediterranean type: no enzyme activity in circulating RBCsG6PDAvoid oxidant stress to RBCsTransfusion if acute anemia is symptomaticHemoglobin Abnormalities (For discussion of hemoglobinopathies, see sections on these topics)EXTRACELLULAR DEFECTS AutoimmuneAutoimmune hemolytic anemiaAlteration in membrane surface antigen (Rh) or abnormal response of B lymphocytes, causing autoantibody formationSpherocytes on blood filmIf Hb < 10 g/dL and reticulocyte count > 10%-nonePositive direct Coombs test to IgG "warm" antibody antibody directed aeainst RBCsSevere anemia may require transfusion"Warm" antibody Prednisone, 2 mg-kg/24 hrPositive indirect Coombs test and antibody detectable in plasmaIVIGDanazolThermal amplitude 35-40degC Some complement (C3b) may be detected on RBCsSplenectomyImmunosuppressivesTests for underlying diseaseFolic acid, 1 mg.;24 hr if chronic"Cold" antibody"Cold" or IgM autoantibody directed against I/i antigen systemAgglutination or rouleaux on blood filmIfHb> 10g/dLand reticulocyte count < 10%-none Positive direct Coombs test to complement (C3b)Severe anemia may require transfusionTests for underlying diseaseAvoid exposure to coldSerology for infectious mononucleosis; anti-i presentIf severe-immunosuppressives and plasmapheresisSerology for Mycoplasma pneumoniae; anti-I presentPrednisone is less effective Splenectomy is not useful Folic acid, 1 mg/24 hr if chronicFragmentationHemolysis DIC, TTP, HUSDirect damage to RBC membraneFragments on blood filmTreat underlying condition Transfusion, but transfused cells also will have shortened life spanExtracorporealDirect damage to RBCFragmentsSupportivemembraneoxygenationmembraneon blood filmTransfusion until ECMO is discontinuedProsthetic heart valveDirect damage to RBC membraneFragments on blood filmFolic acid, 1 mg/24 hr Iron for secondary iron deficiencyBurns-thermal injuryDirect damage to RBC membraneSpherocytes on blood filmSupportive TransfusionHypersplenism Effects of sequestration, i pH. lipases and other enzymes, and macrophages on RBCsThrombocytopenia and neutropeniaTreat underlying condition- cytopenias alt usually mild Splenectomy if complicating other anemia (e.g., thalassemia major) Folic acid, 1 mg/24 hrPlasma Factors Liver diseaseAlteration in plasma cholesterol and phospholipidsTarget cells or speculated RBCs on blood filmTreat underlying condition Abnormal liver function testsTransfusion, but transfused cells also will have shortened life span Folic acid, 1 mg/24 hrAbetalipoproteinemiaAbsence of apo lipoprotein bAcanthocytes on blood filmVitamin E (A, K, and D) Vitamin E deficiency and heightened sensitivity to oxidative damageAbsent chylomicrons, VLDL, and LDLFolic acid, 1 mg''24 hrDietary restriction of triglyceridesInfectionsToxic effects on RBCsAssociated symptoms and signsAntibiotics CulturesSupportiveWilson DiseaseEffect of copper on RBC membrane, usually self-limitedSpherocytes on blood filmPenicillamineCopper, ceruloplasminSupportive Penicillamine challenge and urine copper excretionTransfusion if acute anemia is symptomatic
Malaria
Thalassemia
G-6 PD deficiency
All of above
Hemolytic Anemias