Expert Opinion :
This child has presented with a massive splenomegaly with hepatomegaly, hemolytic facies and intermittent blood transfusion requirement. Thus, one would consider hemolytic anemia as a diagnosis with clinical presentation like a thalassemia intermedia. Common causes such as hemoglobinopathies, hereditary spherocytosis, osteopetrosis, enzyme deficiencies have been ruled out in this child. Also, though child did have low iron or iron studies, serum Ferritin is normal ruling out iron deficiency anemia. Ferritin is usually elevated in hemolytic anemia but may be normal with dyserythropoietic anemias. Also the reticulocyte count is not high for a hemolytic anemia suggesting that destruction of the RBCs seem to be taking place in the marrow itself leading to extramedullary hematopoiesis causing hepatomegaly, splenomegaly and hair on end appearance of the X-Ray skull. A bone marrow done in this child showed congenital dyserythropoietic anemia.