Abstract
Five-year-old girl with immunodeficiency with short-limbed dwarfism is reported. She manifested deficient cell-mediated immunity but normal antibody-mediated immunity. Her T-lymphocyte had maturation arrest at stage I thymocyte.
Two fetal thymus and fetal liver cell transplants were performed on her. Two weeks after the first transplant, mild graft versus host reaction was observed and after four and half months, new HLA antigen was detected. The transplanted fetal stem cells were considered to differentiate in this patient, but no clinical improvement or immunological reconstitution could be observed. The patient continued to deteriorate and expired with interstitial pneumonia five months after the transplantation.
Mild GVH phenomenon and the detectin of new HLA antigen in this patient makes this therapeutic approach promising, and it will be reasonable when histocompatible donors are unavailable.
