A regimen of oral cellulose phosphate, prednisone, and low calcium diet has been reported to be effective in some but not all patients. The prednisone arrests the progression of anemia. Bone marrow transplantation has been reported to be curative in several patients. Generally, the prognosis for survival is poor, and death in the first few months or year from anemia, bleeding, or overwhelming infection is not uncommon. Increased bone density may be discovered incidentally on x- rays.
Most cases appear to represent autosomal dominant inheritance.
The vertebrae show alternating lucent and dense bands. Management should be directed at recognition and treatment of complications, with frequent testing of visual fields and acuity and periodic x- rays of the optic foramina. Transfusion and splenectomy may be useful in some patients. Beighton, P. Inherited Disorders of the Skeleton . Coccia, P. Successful bone marrow transplantation for malignant osteopetrosis.