Unusual onset of chronic graft-versus-host disease after adult living-related liver transplantation from a homozygous donor

Graft-versus-host disease (GVHD) is a rare complication that occurs after living-related liver transplantation (LRLT). This condition usually occurs early after transplantation and is fatal. Although one-way matching between a human leukocyte antigen (HLA)homozygous donor and a haploidentical recipient has been shown to be a significant risk factor for the development of acute GVHD, chronic GVHD has not been clarified. A 50-year-old woman underwent LRLT for primary biliary cirrhosis with her 32-year-old HLA-homozygous son as the donor. The patient developed chronic GVHD 114 days posttransplant, presenting with a skin rash only. Her atypical onset included neither fever nor gastrointestinal symptoms. The patient responded to corticosteroid therapy and is now doing well at home. Careful donor selection and HLA matching before LRLT should be performed to prevent GVHD. However, the risks associated with grafts from homozygous donors may be unavoidable at present because of the shortage of cadaveric donors in Japan.