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 ra...

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Veröffentlicht in:Pediatric hematology and oncology 1999-07, Vol.16 (4), p.361-366
Hauptverfasser: Benito, A., Diaz, M. A., Alonso, F., Fontan, G., Madero, L.
Format: Artikel
Sprache:eng
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Zusammenfassung: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 followup has been uneventful and at this time, 27 months after CBT, immune functions have been recovered.
ISSN:0888-0018
1521-0669
DOI:10.1080/088800199277209