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...

Full description

Saved in:
Bibliographic Details
Published inPediatric hematology and oncology Vol. 16; no. 4; pp. 361 - 366
Main Authors Benito, A., Diaz, M. A., Alonso, F., Fontan, G., Madero, L.
Format Journal Article
LanguageEnglish
Published Philadelphia, PA Informa UK Ltd 01.07.1999
Taylor & Francis
Subjects
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Biological and medical sciences
Bone marrow, stem cells transplantation. Graft versus host reaction
Cord Blood Transplantation
Eosinophilia - therapy
Fetal Blood
Hematopoietic Stem Cell Transplantation
Humans
Immunodeficiencies
Immunodeficiencies. Immunoglobulinopathies
Immunopathology
Infant
Lymphatic Diseases - genetics
Lymphatic Diseases - therapy
Male
Medical sciences
Omenn'S Syndrome
Syndrome
Transfusions. Complications. Transfusion reactions. Cell and gene therapy
Transplantation Conditioning
Unrelated Donor
Human
Immunopathology
Histocompatibility system
Unrelated donor
Leukocyte
Treatment efficiency
Male
Transplantation
Immune deficiency
Case study
Platelet
Newborn
Surgery
Umbilical cord
Online AccessGet full text

Cover

More Information
Summary: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.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Feature-4
content type line 23
ObjectType-Report-1
ObjectType-Article-3
ISSN:0888-0018
1521-0669
DOI:10.1080/088800199277209