A 4 1/2-year-old girl developed severe aplastic anemia in association with dyskeratosis congenita. She was treated by allogeneic bone marrow transplantation following immunosuppression which included high-dose cyclophosphamide. Her clinical course following transplantation was complicated by severe graft-versus-host disease and gastrointestinal toxicity resembling that observed in patients with Fanconi's anemia treated by marrow transplantation. Two other patients with aplastic anemia and dyskeratosis congenita have been reported to develop similar transplant complications. Children with dyskeratosis congenita may be predisposed to the same complications as children with Fanconi's anemia following bone marrow transplantation and require preparative immunosuppressive regimens that do not include high-dose alkylating agents.
BONE MARROW TRANSPLANTATION FOR APLASTIC ANEMIA ASSOCIATED WITH DYSKERATOSIS CONGENITA / V., Conter; F. L., Johnson; Paolucci, Paolo; M., Ruggiero; R. L., Janco. - In: AMERICAN JOURNAL OF PEDIATRIC HEMATOLOGY/ONCOLOGY. - ISSN 0192-8562. - STAMPA. - 10:(1988), pp. 99-102.
BONE MARROW TRANSPLANTATION FOR APLASTIC ANEMIA ASSOCIATED WITH DYSKERATOSIS CONGENITA
PAOLUCCI, Paolo;
1988
Abstract
A 4 1/2-year-old girl developed severe aplastic anemia in association with dyskeratosis congenita. She was treated by allogeneic bone marrow transplantation following immunosuppression which included high-dose cyclophosphamide. Her clinical course following transplantation was complicated by severe graft-versus-host disease and gastrointestinal toxicity resembling that observed in patients with Fanconi's anemia treated by marrow transplantation. Two other patients with aplastic anemia and dyskeratosis congenita have been reported to develop similar transplant complications. Children with dyskeratosis congenita may be predisposed to the same complications as children with Fanconi's anemia following bone marrow transplantation and require preparative immunosuppressive regimens that do not include high-dose alkylating agents.
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