Hereditary pyropoikilocytosis in an infant: A case with early splenectomy

Hereditary pyropoikilocytosis (HPP) is a rare congenital hemolytic disorder characterized by markedly fragile red cell membranes and an unusual sensit ivity to thermal disruption. This communication describes the occurrence of HPP in an infant who developed transfusion dependent hemolytic anemia and required splenectomy at seven months of age. The diagnosis of HPP was estab lished on the basis of red cell morphology and thermal instability test.