An autopsied case with severe combined immunodeficiency after successful engraftment of fetal liver cells

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In the previous paper, we described a report of successful fetal liver transplantation in a 20-month-old boy with severe combined immunodeficiency. In Japan, he is the first case of severe combined immunodeficiency, whose immunity was successfully reconstituted after the transplantation.In this paper, we reported his clinical course and alteration of the immunity after the transplantation.Though the serum immunoglobulin remained very low until his death, the immunological examinations in vitro and in vivo indicated that the cellular immunity was well reconstituted. He discharged 14 months after the transplantation and lived almost uneventfully for a year, while he has been suffered from recurrent bronchopneumonia since 27 months after the transplantation. At the same time, the peripheral lymphocytes showed decreased responses to Con A and PHA. Fifty months after the transplantation, he died of bronchopneumonia. The autopsy revealed generalized cytomegalovirus infection in most of the organs.The transplanted fetal cells were still found in the peripheral blood comfirmed by chromosomal analysis just before his death. The thymus histologically showed marked involution, associated with conspicuous decrease of Hassall's bodies or thymic lymphocytes. The spleen and lymph nodes revealed the normal architecture on light microscope, associated with rare follicular formation.
日本臨床免疫学会の論文