Hereditary spherocytosis in an 18-month-old boy with pancytopenia: A case report

Hereditary spherocytosis (HS) is a familial hemolytic disorder with marked heterogeneity of clinical features, ranging from an asymptomatic condition to a fulminant hemolytic anemia. Although a positive family history of spherocytosis increases the risk for this disorder, it may be sporadic in some cases. In severe cases the disorder may be detected in early childhood, but in mild cases it may go unnoticed until later in adulthood. The case was an 18-month-old boy from Sanandaj, Iran with 3 days decreased activity movement, poor feeding, pallor and urine discoloration since 3 days ego following an episode of fever. He was a case of anemia who was managed conservatively on nutritional supplements. Blood film showed 80% spherocytes, reticulocyte was 0.5%, increased osmotic fragility test and a negative direct Coombs.