RESUMO

Banked unrelated umbilical cord blood matched at 5 of 6 human leukocyte antigen loci was used to reconstitute the immune system in 2 brothers with X-linked lymphoproliferative syndrome and 1 boy with X-linked hyperimmunoglobulin-M syndrome. Pretransplant cytoreduction and posttransplant graft-versus-host prophylaxis were given. Hematopoietic engraftment and correction of the genetic defects were documented by molecular techniques. Two years after transplantation, all 3 patients have normal immune systems. These reports support the wider use of banked partially matched cord blood for transplantation in primary immunodeficiencies.

Assuntos

Sangue Fetal/citologia , Transplante de Células-Tronco Hematopoéticas/métodos , Transtornos Linfoproliferativos/terapia , Ligante de CD40/genética , Criança , Pré-Escolar , Análise Mutacional de DNA/métodos , Doença Enxerto-Hospedeiro/prevenção & controle , Humanos , Lactente , Transtornos Linfoproliferativos/genética , Transtornos Linfoproliferativos/imunologia , Masculino , Linfócitos T/imunologia