Prednisolone-responsive Malignant Osteopetrosis: a case reveiw

ABSTRACT

The authors presented a 17 months old female with malignant osteopetrosis, who was admitted to the Pediatric Department of Kyung Hee University Hospital on May, 1981 because of epistaxis and growth retardation. Diagnosis was made by typical clinical manifestations, hematologic and radiologic findings, such as frontal bossing, opened anterior fontanel, exophalmoses, strabismus, optic atropy, marked hepatosplenomegaly, severe anemia, thrombocytopenia, reticulocytosis and typical mask sign and sun burst sign at the skull X-ray, wide dense ribs with fracture at the vertebrae, clubbings at the distal parts of the femurs and radii and at the proximal parts of the tibias and humerii transverse bands at the metaphyses, and finally increased density of the entire skeleton on the bone scanning and marked decrease uptake of 99mTc on the bone marrow scanning. Prednisolone was administrated orally, resulted in gradual improvement of hematologic abnormalities to the normal range, associated wited with increased cellularity of the bone marrow and increased uptake of 99mTc and decreased spleen size on the bone marrow scanning after one month of therapy.