2012 Volume 58 Issue 5 Pages 704-709
Allogeneic hematopoietic stem cell transplantation is the only curative treatment for severe combined immunodeficiency (SCID). Recently, we treated two children with SCID (X-linked SCID and adenosine deaminase (ADA) deficiency) who underwent bone marrow transplantations (BMT) from HLA-identical minor mismatch donors. Conditioning was not performed in the patient with X-linked SCID, whereas reduced-intensity conditioning was done in the patient with ADA deficiency. In this study, antigens on red blood cells (RBC) and chimerism of subpopulations of white blood cells (WBC) were sequentially examined. Immunological status of both patients was completely improved. The patient with X-linked SCID showed that RBCs were derived from the donor, and subpopulations of WBCs except for T cells had mixed chimerism at 2.5 years after BMT. The patient with ADA deficiency showed that all cells were derived from the donor at one year after BMT. The patient with X-linked SCID requires follow-up for a long period.
