hemolytic anemia excessive destruction of red cells acute hemolytic anemia chronic hemolytic anemia...
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Hemolytic anemia
Excessive destruction of red cells
Acute Hemolytic anemia
Chronic Hemolytic anemia
Congenital
Acquired : Immune Non-immune
Classification of hemolytic anemia
- Intravascular hemolytic anemia :
- Turbolent and Marsch hemoglubinuria
-Fibrin 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 CellCell Destruction
- Decreased erythrocyt life span
- Increased catabolism of heme.
1- Hemoglbinemia
2- Hemoglobinuria
3- Hemosiderinuria
4- Methemalbumineuria
6- Reduced serum hemopexin level
-Fall in blood hemoglobin level at a rate greater than 1.0 g/dl/week
5- 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 Disorders
1- 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” ) Test
The 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 Test
The 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 anemia
Congenital
Acquired : Immune Non-immune
Etiology of Hemolytic anemia
1- Congienital :
Defect of Hemoglobin synthesis and structureThalassemiaSickle cell diseaseUnstable hemoglobins
Membrane defectsHereditary spherocytosisHereditary elliptocytosis
Red cell enzyme defectsG6PD deficiencyPyruvate kinase deficiency
2- 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 immunodeficiency
3- 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