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