A 3-year-old child showed progressive anemia, jaundice, and failure to thrive. Examination revealed pallor and splenomegaly, while the peripheral smear demonstrated normoblasts and small, round, intensely stained red cells. The plausible diagnosis is________________?
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Answer: A. Here ditary spherocytosis
Explanation
Hereditary spherocytosis presents with hemolytic anemia, jaundice, and splenomegaly. The peripheral blood smear shows characteristic small, round, hyperdense spherocytes. Defective red blood cell membrane proteins cause premature destruction in the spleen. This condition is an inherited membrane skeleton defect involving spectrin orankyrin. Increased splenic destruction of fragile spherocytes leads to chronic hemolytic anemia. Splenectomy is curative for the anemia because it removes the site of destruction.
Patients often present with pigmented gallstones due to chronic bilirubin breakdown. Early diagnosis helps manage complications associated with severe hemolytic crises. Hematology consultations guide supportive care and surgical intervention decisions. Thalassemia features hypochromic microcytic red blood cells and hemoglobin synthesis defects. Sickle cell anaemia presents with abnormal hemoglobin S causing crescent-shaped cells. Vitamin B12 deficiency anaemia shows megaloblastic changes and macrocytic red cells.
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