Hemolytic Anaemias Classification Diagnosis And Treatment

Hemolytic Anaemia

• The loss of red cells by hemolysis or destruction are main causes hemolytic anaemia. The life span of RBCs is 90 to 120 days in circulation.
• The destroyed RBCs are re­placed by new red cells formed by the bone marrow. If the rate of destruction of RBCs is more than the rate of replenishment, then anaemia occurs.
• There is jaundice due to rise in bilirubin generated by destruction of RBCs. There are symptoms of anaemia like fatigue and dyspnoea.

Hemolytic Anaemia jaundice

• There is passage of red brown urine (hemoglobin­uria).
• There is history of exposure to drugs or toxins. Usually there is splenomegaly.

Lab tests

• There is evidence of hemolysis on examination of blood smears.
• Reticulocyte count is elevated due to erythroid hy­perplasia of the bone marrow.
• Reticulocytes are also elevated in patients with blood loss, myelophthisis, decrease of red blood cells in cir­culation due to any cause.

Red blood cell morphology IS altered as follows

• · Spherocytes in hereditary spherocytosis.
• · Target cells in hemoglobin disorders ­thalassemias, hemoglobin Sand C.
• · Schistocytes in microangiopathy, intravascular prosthesis.
• · Sickle cells in sickle cell syndromes.
• · Acanthocytes and spur cells in severe liver dis­ease.
• · Agglutinated cells in cold agglutinin disease.
• · Heinz bodies in oxidant stress.

Classification of hemolytic anaemias

• Hemolytic anaemia may be due to intracorpuscular defect or extracorpuscular factors.
• The intracorpuscular (defect is within the RBC) causes are enzyme defects, hemoglobinopathies, hereditary spherocytosis.
• The extracorpuscular factors are liver disease – spur cell .anaemia, hypersplenism, immune complexes, in­fections, toxins, microangiopathy.

Treatment of Hemolytic Anaemia

• Splenectomy reduces the hemolysis and im­proves RBC survival.
• Splenectomy is done by laparoscopy for low risk. Folic acid 1 mgjday is given before splenectomy.

HEMOLYTIC ANAEMIA DUE TO DEFECTS IN HEX­OSE – MONOPHOSPHATE SHUNT

• When RBC’s are exposed to a drug or toxin which generates free radicals, then glucose metabolism via hexose monophosphate shunt starts to occur. This protects the RBC membrane from oxidation and de­struction.
• In G6PD deficiency the hexose monophosphate shunt becomes defective and hemoglobin precipitates within the RBC forming Heinz bodies.
• There is no treatment but the disease is self remit­ting.
• Oxidant drugs, infections, and Fava beans should be avoided.
• Drugs causing hemolysis in G6PD deficiency patients­Primaquine, Sulphonamides, Nitrofurantoin, Nalidixic aCid, Furazolidone, Vitamin K, dapsone, doxorubicin etc.