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The Journal of Bone and Joint Surgery (American) 79:1726-30 (1997)
© 1997 The Journal of Bone and Joint Surgery, Inc.

Case Report

Bone-Marrow Transplantation in Sickle-Cell Disease. Effect on Osteonecrosis: A Case Report with a Four-Year Follow-up*

P. HERNIGOU, M.D.{dagger}, F. BERNAUDIN, M.D.{dagger}, P. REINERT, M.D.{dagger}, M. KUENTZ, M.D.{dagger} and J. P. VERNANT, M.D.{dagger}, CRETEIL, FRANCE

Investigation performed at Hôpital Henri Mondor, Creteil

The first 150 words of the full text of this article appear below.


   Introduction
 
Sickle-cell disease involves both hematological and osseous abnormalities because it affects the two major functions of bone tissue: hematopoiesis and osteogenesis. Symptoms include dactylitis, painful vaso-occlusive crises, splenic reticuloendothelial dysfunction (also called functional asplenia), and multiple-organ damage and failure in children and adults. It is probably the most common cause of avascular necrosis worldwide5; in series of adult patients, the reported prevalence of involvement of the femoral head was 30 per cent (ninety-five of 320 hips)13 and that of the humeral head was 48 per cent (106 of 220 shoulders)11.

Osteopetrosis is another disease that is associated with both hematological and osseous abnormalities. Walker, in the mid-1970s, performed a series of experiments on osteopetrotic mice and found that it was possible to correct both types of abnormalities simultaneously with use of a bone-marrow allograft26-29.

The use of bone-marrow transplantation for the treatment of sickle-cell disease was reported . . . [Full Text of this Article]


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