Therapy

Radiation in a dose of 40-50 cGy is indicated for patients with single or multiple osteosclerotic lesions in a limited area. More than half of the patients show substantial improvement of neuropathy. Of our 13 patients who did not respond to radiation, 9 received less than 4000 cGy. The improvement may be slow and may not be apparent for 6 months or longer. Some patients continue to improve for 2-3 years after radiation therapy.

Systemic therapy is necessary if the patient has widespread osteosclerotic lesions. Melphalan and prednisone were given to 48 of our patients, and improvement was noted in 44%. Combination chemotherapy induced responses in 27% of 15 treated patients. Prednisone or dexamethasone as a single agent was given to 41 of our patients, and there was an apparent improvement in only 15%. Plasma exchange, intravenous immunoglobulin, cyclosporine, and aza-thioprine were ineffective.

Autologous stem cell transplantation after high-dose melphalan should be seriously considered for patients younger than 70 years with widespread osteosclerotic lesions. The stem cells should be collected before the patient is exposed to alkylating agents because they will damage the hematopoietic stem cells. The mortality associated with the procedure is only 1-2%.67-70

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