Which of the following is not seen in'Hereditary Spherocytosis:
Answer is A (Direct Coomb's Positive)Coomb's Negative Haemolytic Anaemia.Coomb's test is impoant to distinguish Hereditary Spherocytosis from Autoimmune haemolysis which is another common cause of Spherocytosis. Hereditary Spherocytosis is a coomb's negative haemolytic anemia (non-immune mediated) characterized clinically by anaemia, jaundice, splenomegaly and pigment gall stones. Typical Laboratory findings include spherocytosis (abundant spherocytes on peripheral smear) and increased osmotic fragility. There is no definitive treatment to correct the primary defect in the membrane cytoskeletal structure. Splenectomy remains the treatment of choice for severe cases of Hereditary Spherocytosis (since Spleen is the primary site of destruction of red blood cells and transit through the splenic circulation makes them more spherocytic thereby accelerating their death.
Direct Coomb's Positive
Increased Osmotic Fragility
Splenomegaly
Gall Stones