Abstract
Adenosine deaminase(ADA) activity and immunological functions following successfull bone marrow transplantation(BMT) was evaluated for 3 years in a 3-year-old boy with ADA deficieny and severe combined immmunodeficiency(SCID) who was diagnosed at the age of 1 month and recieved BMTs three times during the following 3 months from a HLA-identical brother. On 12th day of posttransplantation, ADA activities of lymphocytes and red cells, which were below one-tenth of the normal levels, began to increase and reached within the normal level on the 19th day. Simultaneously, dATP in red cells and dAdo in urine reduced significantly. The persistent chimerism of donor red cells disappeared spontaneously 2 years later, when ADA activity in red cells had decreased further to undetectable level. On the other hand, B-cell functions began to recover about one month after transplantation. T-lymphocytes with the surface markers of OK3, OK4, LeulO and Leu7 increased in number within the normal levels, although T-lymphocyes with positive OKT8 antigen delayed to appear untill about 2 months later. The in vitro responsiveness to PHA and Con-A mitoges started to recover slowly on about 3rd month after 3rd BMT. The patient is still quite healthy and developes very well without any GVHD.
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Hyodo, Y., Itoh, R., Kurozumi, H. et al. 59 IMMUNOLOGICAL AND METABOLIC RECONSTRUCTION FOLLOWING SUCCESSFULL BONE MARROW TRANSPLANTATION FROM A HLA-IDENTICAL SIBLING IN AN INAFANT WITH ADENOSINE DEAMINASE DEFICIENCY AND SEVERE COMBINED IMMUNODEFICIENCY. Pediatr Res 24, 121 (1988). https://doi.org/10.1203/00006450-198807000-00083
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DOI: https://doi.org/10.1203/00006450-198807000-00083