Greffe de moelle dans l’anémie de Fanconi : à propos de sept cas

Objects.-Follow-up of patients with Fanconi's anemia treated in our unit and review of the literature concerning bone marrow transplantation in Fanconi's anemia. Patients and methods.-Ten patients were followed in our unit for 20 years. We summarize their clinical features, treatment and clinical course. Results.-Among the ten patients, seven received allogeneic marrow transplantation. Only two patients are still alive. Two transplanted patients died from complications shortly after the transplantation. Three other patients died later after the transplantation, two of them from oropharyngal carcinomas. Discussion.-The 5-year survival is about 70% in the transplantation with an HLA-identical sibling donor; it is only about 30% if the donor is an HLA-matched unrelated or mismatched related patient. Furthermore, retrospective studies have shown that the long-term outcome of carcinoma is a major complication after the transplantation. Conclusion.-Our series of patients with Fanconi's anemia reflects fairly faithfully the complications encountered in this disease. Although the improvement of the graft technique may decrease the rate of death due to transplantation, the long-term development of solid tumors remains a problem for which no solution has been suggested up to now. (C) 2001 Editions scientifiques et medicales Elsevier SAS.