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Hemolytic anemia Excessive destruction of red cells Acute Hemolytic anemia Chronic Hemolytic anemia Congenita l Acquired : Immune Non-immune

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  • Hemolytic anemiaExcessive destruction of red cellsAcute Hemolytic anemiaChronic Hemolytic anemiaCongenitalAcquired : Immune Non-immune

  • Classification of hemolytic anemia- Intravascular hemolytic anemia : - Turbolent and Marsch hemoglubinuriaFibrin deposetion : DIC , TTP , UHS (Microangiopathic )Complement lysis : PNH , PCH , and Non - compatible blood transfusion- Toxin : colestidium velshay bacter- Extravascular hemolytic anemia RBC desruction by :- Immune Liver- Membran defect Spleen Other tissues- Enzymppathies: G6PD D. , Pyrovate Kynase.d.

  • Laboratory Signs of Accelerated Red Cell Destruction- Decreased erythrocyt life span- Increased catabolism of heme.1- Hemoglbinemia2- Hemoglobinuria3- Hemosiderinuria4- Methemalbumineuria

    6- Reduced serum hemopexin levelFall in blood hemoglobin level at a rate greater than 1.0 g/dl/week5- Absent Haptoglobin

  • In the chronic hemolytic anemia

    - quite severe anemia can be tolerated with symptoms only arising on exercise.-The patients jaundiced but this is often mild.

    The urine is dark especially after standing

    The spleen tend to be enlarged to degree of the underlying cause of the hemolysis .

    - Leg ulcer usualy over the lateral malleolus

  • Immune-Mediated Hemolytic Disorders1- IgG warm autoantibodies: IgG bind to RBC at 37c but fail to agglutinate the RBC.2-Cold agglutinins: almost always are are of the IgM subtype and clump RBC at cold temperatures.3- Donath-Landsteiner ( IgG) antibodies. Antibodies bind to RBC membranes in the cold and activate the Hemolyitic complement cascade .

  • Laboratory features of hemolytic anemia- Raised level of unconjugated bilirubin- Reticulocytosis- Urine hemosiderinuria- Red cell survival studies with C-labelled- Erythroide hyperplasia in bone marrow- Examination of peripheral blood smear- Direct antiglobulin test ( Coombs test )- Absent haptoglubin- Hemoglubinemia and hemoglubinuria

  • The Antiglobulin ( Coombs ) TestThe test for routine detection of immunohemoiytic anemia , used most widely is the direct antiglobulin or Coombs test.

    Positive results indicated thet the red cells are coated with IgG and / or complement components, especially C3.

    2- 5 % of patients with immuno-hemolytic disease have negative test results because the amount of globulin on the cell surface is below the detection limits.

  • The Osmotic Fragility TestThe Osmotic F.T. is a measure of the resistance of erythrocytes to hemolysis by osmotic stress .

    The test consists of exposing red celle to decreasing strengths of hypotonic saline Solutions and measuring the degree of hemolysis.

    Normally , hemolysis begin 0.45 to 0.50 g/dl and at which it is complete normally 0.30 to 0.33 g / dl.

    Increased fragility is indicated by a shift of the curve to the left or high value for median corpuscular fragility ( MCF ).

  • Chronic Congenital Hemolytic Anemia- Various degree of anemia- Jaundice- Crises- Splenomegaly- Cholelithiasis- Leg Ulcers- Skeletal Abnormalities

  • Hemoglubin defects Defects of synthesis : the thalassemias syndrome.- Defects of structure : Sickle cell disease.

  • Acute Hemolytic anemiaCongenitalAcquired : Immune Non-immune

  • Etiology of Hemolytic anemia1- Congienital :

    Defect of Hemoglobin synthesis and structureThalassemiaSickle cell diseaseUnstable hemoglobinsMembrane defectsHereditary spherocytosisHereditary elliptocytosisRed cell enzyme defectsG6PD deficiencyPyruvate kinase deficiency2- Acquired (Non-Immune)3- Acquired ( immune )

  • Congenital

  • Etiology of Warm antibody type autoimmune Hemolytic A. Idiopatic (primary)Secondary :1- Lymphoprolifrative diseasee, CLL ,NHL.2- Connective tissue diseases ,SLE,variable immunodeficiency3- Immune deficiency disorders,AIDS, common variable imm.d.4- Druges induced immuno-hemolytic anemia:penicilline , Quinidine , Methyldopa , Cephalosporine

  • Treatment of warm autoantibodu H.A.If mild ,no specific therapy may needed.Treatment of secondary causes ,underling diseases.When treat underling diseases Unsuccessful:- Folic acid and Vit B12- RBC transfusions- Corticosteroids- Splenoctomy- IV immunoglobulin Immunosupressive therapy- Danazol and Vinca alkaloids

  • Treatment of warm autoantibodu H.A.If mild ,no specific therapy may needed.Treatment of secondary causes ,underling diseases.When treat underling diseases Unsuccessful:- Folic acid and Vit B12- RBC transfusions- Corticosteroids- Splenoctomy- IV immunoglobulin Immunosupressive therapy- Danazol and Vinca alkaloids

  • - Folic acid and Vit B12- RBC transfusions- Corticosteroids- Splenoctomy- IV immunoglobulin Immunosupressive therapy- Danazol and Vinca alkaloids

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