Successful unrelated umbilical cord blood transplantation in a child with Omenn's syndrome

Omenn's syndrome is a variant of combined immunodeficiency disease (CID). L ike other CID forms, it causes death unless the patient receives a bone mar row transplant (BMT). Previous reports have shown that BMTs from unrelated donors in Omenn's syndrome have very poor results, with a high rate of infe ctions during transplantation and graft rejection, when compared with trans plants from related donors or patients with other CTD. This study discusses the case of a 19-month-old child with Omenn's syndrome, who received an un related cord blood stem cell transplant (CBT). Donor and recipient had 1 HL A-Ag mismatched on HLA-B. Symptomatology improved early after CBT: The chil d achieved leukocytes and platelet engraftment and urns discharged on day 34. His follow-up has been uneventful and at this time, 27 months after CBT immune functions have been recovered.