Haemolytic Anaemia in Osteopetrosis: A Report of Two Cases

Abstract

The anaemia so frequently found in cases of osteopetrosis in children has been generally regarded as being due to poor development of the bone marrow cavity by failure of absorption and organiza- tion of primitive chondro-osteoid tissue (Warkany, 1959; Clifton and Frank, 1959; Aegerter and Kirk- patrick, 1958). In many cases, however, the severity of the anaemia has not corresponded with the radiological appearances of the medullary cavity, and alternative theories have been put forward to explain this discrepancy. Some workers have suggested that the anaemia is due to a primary bone marrow defect (McCune and Bradley, 1934), and others (Creveld and Heybroek, 1940) have postu- lated that the bone lesions and the anaemia are both part of a common developmental mesenchymal disturbance. Hepatosplenomegaly, a common feat- ure of this disorder, has been taken to indicate increased extramedullary erythropoiesis, and in view of this, splenectomy in cases of osteopetrosis is considered therapeutically unsound.