HAEMOLYTIC ANEMIA

HAEMOLYTIC ANEMIA

It's caused of increasing destruction of red cells reducing of lifespan.

NORMAL RED CELLS LIFE TIME IS 120 DAYS.

INHERTED CAUSES OF HAEMOLIC ANEMIA:

HEREDITRY SPHEROCYTOSIS.

HEREDITARY ELLIPTOCYTOSIS.

ABNORMALITIES OF HAEMGLOBINE:

THALASSAEMIA.

SICKLE-CELL DISEASE.

METABOLIC DEFCTS:

GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENY.

PYRUVATE KINASE DEFICIECY.

ACQUIRED CASUSES HAEMOLTIC ANEMIA:

IMMUNE SYSTEM DISORDER:

Autoimmune  hemolytic anemia.

Hemolytic transfusion reactions.

Miscellaneous:

Infection.

Chemicals – drugs.

Hypersplenism.

 OTHER RESEONE OF HEMOLTIC ANEMIA:

March hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria.

Microangiopathic hemolytic anemia.

MACROPHAGES IN THE RETICULOENDOTHEL-IAL SYSTEM DESTRUCTED RED CELLS FROM THE CIRCULATION.

INHERITED HAEMOLTIC ANEMIA:

SPHEROCYTOSIS:

It's heredity disease most commune inherited in northern Europeans.

Caused by deficiency of one protein called spectrin, this protein very important for red cell membrane formation.

Spectrin deficiency leads to deformation of red cell shape, which become spherical in shape these cells are more rigid and they are less deformable than normal red sell.

That pathological shape lead to be destroyed by spleen prematurely.

SYMPTOMS OF HEREDITARY SPHEROCYTOSIS ANEMIA:

Jaundice – symptoms of anemia – splenomegaly – legs ulcers.

CHROCNI HEMOLYSIS LEADS TO PIGMENT GALLSTONES FROMATINS.

LABORATORY FINDINGS:

Blood count:

Mild anemia with reticulocytosis.

Blood film:

Spherocytes and reticulocytes.

Diagnosis made by:

Increase red sell osmotic fragility when placed in hypotonic solution.

                          

SPHEROCYTOSIS ANEMIA MANAGEMENT:

In all cases should perform splenoctamy and all patient shoul resive pneumococcal vaccine with long term penicillin also for prophylactics.