Successful unrelated umbilical cord blood transplantation in a child with Omenn's syndrome.
Omenn's syndrome is a variant of combined immunodeficiency disease (CID). Like other CID forms, it causes death unless the patient receives a bone marrow transplant (BMT). Previous reports have shown that BMTs from unrelated donors in Omenn's syndrome have very poor results, with a high rate of infections during transplantation and graft rejection, when compared with transplants from related donors or patients with other CID. This study discusses the case of a 19-month-old child with Omenn's syndrome, who received an unrelated cord blood stem cell transplant (CBT). Donor and recipient had 1 HLA-Ag mismatched on HLA-B. Symptomatology improved early after CBT. The child achieved leukocytes and platelet engraftment and was discharged on day +34. His follow-up has been uneventful and at this time, 27 months after CBT, immune functions have been recovered.
Benito
A
A
Department of Pediatric Hematology and Oncology, Hospital Infantil Niño Jesús, Autonomous University of Madrid, Spain.
Diaz
M A
MA
Alonso
F
F
Fontán
G
G
Madero
L
L
D004802:Eosinophilia / Q000628:therapy*; D005312:Fetal Blood; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D008206:Lymphatic Diseases / Q000235:genetics / Q000628:therapy*; D008297:Male; D013577:Syndrome; D019172:Transplantation Conditioning