Abstract
A year-old male with SCID was transplanted with fetal liver & thymus from a female fetus removed by hysterotomy from his mother at 16 weeks gestation. The fetus differed from the patient in both HL-A haplotypes. Four days after transplantation, a GvsH reaction appeared: fever, diarrhea, maculopapular rash & pos. skin biopsy. By 14 days, liver enzymes & bilirubin were elevated. Max. values: bilirubin 30mg% (tot.), 19mg% (dir.), SGOT 405U & SGPT 350U.
Chimerism was shown by total change in lymphocyte HL-A antigens T cells rose from 5% to 75%, but did not respond to in vitro stimulation by mitogens, antigens & allogeneic cells to date, 7 ½ mos. after transplantation. Immunoglobulins remained essentially absent. Following anti-thymocyte globulin & steroids, bilirubin remains around 10-14mg% (tot.) & 3-8mg% (dir.) & the enzymes have decreased. Liver biopsy 5 mos. after transplantation showed no necrosis, intact lobular architecture, patchy canalicular & hepatocellular bile stasis. Portal bile ducts were totally absent. There were small lymphocytic portal infiltrates. No plasma cells, hematopoietic cells, or fibrosis were seen. No virus was isolated.
The observations show that these fetal cells, capable of effecting a severe GvsH reaction in vivo, repeatedly did not respond to various stimuli in vitro before or after transplantation. The observations also suggest that a GvsH type reaction may be one cause of the “disappearing bile duct syndrome.”
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Cohen, F., Raghunath, R., Zmijewski, C. et al. “DISAPPEARING BILE DUCT SYNDROME,” RESULT OF A GRAFT VERSUS HOST (GvsH) REACTION, AFTER FETAL LIVER AND THYMUS TRANSPLANTATION FOR SEVERE COMBINED IMMUNODEFICIENCY DISEASE (SCID). Pediatr Res 11, 485 (1977). https://doi.org/10.1203/00006450-197704000-00692
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DOI: https://doi.org/10.1203/00006450-197704000-00692