Abstract

INTRODUCTION: Graft versus host disease (GVHD) is a rare, life-threatening complication of liver transplantation that may affect the skin, GI tract and bone marrow. Donor T-lymphocytes remaining in the transplanted graft primarily target the recipient causing organ injury. Presentation may be non-specific and mimic other post-transplant complications, leading to delay in diagnosis and a mortality rate of greater than 80%. There is no consensus regarding optimal management of GVHD in the setting of liver transplantation. CASE DESCRIPTION/METHODS: A 65-year-old male with a history of NASH cirrhosis underwent deceased-donor liver transplant. Induction therapy was given with basiliximab and solumedrol then immunosuppression was maintained with tacrolimus, mycophenolate mofetil (MMF) and prednisone. Three weeks post-transplant, diarrhea developed and MMF was dose-reduced. Given persistence of diarrhea, fever, neutropenia and bilateral lower extremity petechial rash, additional work-up was performed. Imaging revealed colonic inflammation and subsequent flexible sigmoidoscopy demonstrated diffuse inflammation in the sigmoid. Biopsies of the colonic mucosa and lower extremity skin rash were consistent with GVHD. Peripheral blood confirmed the presence of donor chimerism. The patient was started on empiric antibiotics for febrile neutropenia, received granulocyte stimulating factor for leukopenia, intravenous steroids, and oral budesonide. MMF was initially discontinued but later resumed at increased dose; tacrolimus was continued. The patient's condition continued to deteriorate with diffuse mucosal GI bleeding. He received antithymocyte globulin and etanercept but his illness progressed secondary to Enterococcus bacteremia leading to septic shock. The patient died 8-weeks post-transplant in the setting of multiorgan failure. DISCUSSION: While rare, GVHD after liver transplantation should be considered in patients with new onset, unexplained symptoms. By virtue of its rarity after liver transplantation, data are sparse and management decisions are challenging. This case illustrates the need for increased awareness and standardized treatment guidelines. Management decisions are currently based on small case series often with discordant strategies including increasing or decreasing immunosuppression exposure. Global registries and multicenter collaboration will be essential to increase knowledge and improve outcomes.

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