Haemolytic Anemia by Dr. Sookun Rajeev Kumar
- 1. HAEMOLYTIC ANEMIA Dr. Sookun Rajeev K (MD) Dept of General Medicine Anna Medical College
- 2. DEFINITION: •Hemolytic anemias are caused by decreased red blood cell survival from increased destruction of the cells. •The destruction may be either in the blood vessels (intravascular) or outside the vessels (extravascular),which generally means inside the spleen.
- 3. CLASSIFICATION 1. Congenital / Hereditary 2. Acquired
- 4. ETIOLOGY The hemolytic anemias may either be: CHRONIC as in sickle-cell disease, paroxysmal nocturnal hemoglobinuria and hereditary spherocytosis OR ACUTE such as in drug induced hemolysis, autoimmune hemolysis or glucose 6-phosphate dehydrogenase deficiency.
- 5. HEMOLYTIC ANEMIA Causes •INTRACORPUSCULAR HEMOLYSIS Membrane Abnormalities Metabolic Abnormalities Hemoglobinopathies •EXTRACORPUSCULAR HEMOLYSIS Nonimmune Immune
- 6. HEMOLYTIC ANEMIA Membrane Defects •Microskeletal defects •Hereditary spherocytosis •Membrane permeability defects •Hereditary stomatocytosis •Increased sensitivity to complement •Paroxysmal nocturnal hemoglobinuria
- 7. CLINICAL PRESENTATION The usual symptoms of anemia are present based on the severity of the disease, not necessarily the etiology. Fatigue and weakness occur with mild disease. Dyspnea and later confusion with more severe disease. Hemolysis is often associated with jaundice and dark urine as well.
- 8. CLINICAL PRESENTATION Fever Chills Chest pain Tachycardia Backache may occur if the intravascular hemolysis is particularly rapid.
- 9. DIAGNOSIS •Hemolytic anemias generally have a normal MCV but the MCV may be slightly elevated because reticulocytes are somewhat larger than molder cells. •The reticulocyte count is elevated. •The Lactate Dehydrogenase (LDH) and indirect bilirubin are elevated. Bilirubin levels above four are unusual with hemolysis alone.
- 10. DIAGNOSIS •The peripheral smear shows fragmented cells and the haptoglobin may be low with intravascular hemolysis. The haptoglobin level is often unreliable. •Hemoglobin may be present in the urine when intravascular hemolysis is sudden and severe because free hemoglobin spills into the urine. •There should not be bilirubin in the urine because indirect bilirubin is bound to albumin and should not filter at the glomerulus.
- 11. TREATMENT •Transfusion is needed as in all forms of anemia when the hematocrit becomes low. •Hydration is, in general, useful to help prevent toxicity to the kidney tubule from the free hemoglobin. •Folic Acid 5mg weekly, prophylaxis life long •Splenectomy •Blood transfusion in Acute, severe hemolytic crisis