Hemolytic Anemias

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Hemolytic Anemias. Defined as those anemias result from an increased in the rate of red cell destruction. The red cell destruction is usually removed extravascular by macrophages of reticuloendothelial system (RE) [BM, liver & spleen]. Acute destruction or chronic destruction - PowerPoint PPT Presentation

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  • Hemolytic AnemiasDefined as those anemias result from an increased in the rate of red cell destruction.The red cell destruction is usually removed extravascular by macrophages of reticuloendothelial system (RE) [BM, liver & spleen].Acute destruction or chronic destructionExtravascular or Intravascular hemolysisHereditary or Acquired hemolytic anemiaSJAOUNI, KAU

  • SJAOUNI, KAU

    Classification of hemolytic anemiasHereditaryAcquiredMembraneHereditary spherocytosis, hereditary elliptocytosisImmuneAutoimmune Warm antibody typeMetabolism Cold antibody typeG6PD deficiency, pyruvate kinase deficiencyAlloimmune Hemolytic transfusion reactionsHemoglobinThalassemia disorders Hemolytic disease of the newborn Allografts, especially marrow transplantationAbnormal (Hb S, Hb C, unstable);Drug associatedRed cell fragmentation syndromes Arterial grafts, cardiac valves

  • SJAOUNI, KAUG6PD, glucose-6-phosphate dehydrogenase; Hb, hemoglobin.

    cont. of Classification of hemolytic anemiasHereditaryAcquiredMicroangiopathic Thrombotic thrombocytopenic purpura Hemolytic uraemic syndrome Meningococcal sepsis Pre-eclampsia Disseminated intravascular coagulationMarch hemoglobinuriaInfectionsMalaria, clostridiaChemical and physical agentsEspecially drugs, industrial/domestic substances, burnsSecondaryLiver and renal diseaseParoxysmal nocturnal hemoglobinuria

  • DiagnosisHistoryClinical featuresLaboratory findingsCBC (Hb & MCV), Plt, (WBC & diff)ReticBlood filmSpecific testsSJAOUNI, KAU

  • CBCWBC8.310^3/LNE%64.5 %LY%27.6 %MO%5.1 %EO%2.6 %BA%0.2 %NRBC%0.0 %NE#5.310^3/LLY#2.310^3/LMO#0.410^3/LEO#0.210^3/LBA#0.010^3/LNRBC#0.010^3/L

    RBC4.9410^6/LHGB14.3g / dLHCT40.8 %MCV82.6fLMCH28.9pgMCHC35.0g / dLRDW12.8 %

    PLT26810^3/LMPV7.5fL

    RET%RET#IRFMRVSJAOUNI, KAU

  • Laboratory findings in Hemolytic AnemiaFeatures of increased red cell destruction: S. bilirubin, urine urobilinogen breakdown, fecal stercobilinogen, s. haptoglobins absent.Feature of red cell productionsReticulocytosisBM erythroid hyperplasiaDamaged red cellMorphologyOsmotic fragility & autohemolysisRed cell survivalSJAOUNI, KAU

  • Autoimmune Hemolytic Anemia (AIHAs)Caused by antibody production by the body against its own red cells.Characterized by a positive direct antiglobulin test (DAT) called Coombs testDivided into warm and cold according Abs react at 37oC or 4oC.SJAOUNI, KAU

  • SJAOUNI, KAU

    Autoimmune Hemolytic AnemiaWarm TypeCold TypeIdiopathicIdiopathicSecondary: Systemic lupus erythematosus, other connective tissue disorders

    chronic lymphocytic leukaemia

    malignant lymphoma

    ulcerative colitis

    ovarian teratoma

    drugs, e.g. methyldopaSecondary: Mycoplasma pneumonia

    infectious mononucleosis

    malignant lymphoma

    paroxysmal cold hemoglobinuria: rare; may be primary or associated with infection

  • Cold Autoimmune Hemolytic AnemiaIn these syndromes the antibodies whether:Monoclonal: Idiopathic cold agglutination lymphomas.Polyclonal as infection: Infectious mononucleosis, Mycoplasma pneumoniaPatient may have a chronic hemolytic anemia aggravated by cold. Sometimes associated with intravascular hemolysis.Laboratory:Similar like warm AIHALess spherocyte, more red cell agglutinationDAT reveals complement (C3d)SJAOUNI, KAU

  • SJAOUNI, KAUG6PD, glucose-6-phosphate dehydrogenase.

    Causes of intravascular hemolysisMismatched blood transfusion (usually ABO)G6PD deficiency with oxidant stressRed cell fragmentation syndromesSome autoimmune hemolytic anemiasSome drug- and infection-induced hemolytic anemiasParoxysmal nocturnal hemoglobinuriaMarch hemoglobinuriaUnstable hemoglobin

  • Causes of hereditary hemolytic anemia.SJAOUNI, KAU

  • INHERITANCE

    AUTOSOMAL SEX-LINKEDSJAOUNI, KAU

  • SJAOUNI, KAUSex-linked Inheritance

  • Glucose-6-Phosphate Dehydrogenase (G6PD)DeficiencySex-linked hereditary hemolytic anemiaAcute intravascular hemolysis due to oxidant stress.Episodes of acute hemolytic anemiaMost patients are asymptomatic4 Clinical syndromes: oxidative stress-induced hemolysis by certain drugs, infection and other illness.Favism (fresh and uncooked beans are more dangerous than dried cooked ones.Neonatal jaundiceChronic hemolytic anemiaSJAOUNI, KAU

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