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.
Department of Pediatric Hematology and Oncology, Hospital Infantil Niño Jesús, Autonomous University of Madrid, Spain.
A
A
Benito
M A
MA
Diaz
F
F
Alonso
G
G
Fontán
L
L
Madero
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