M A Elfeki1, P V Genco1, S Pungpapong1, R E Nakhleh1, J H Nguyen1, D M Harnois2. 1. Department of Transplantation, Mayo Clinic Florida, Jacksonville, Florida. 2. Department of Transplantation, Mayo Clinic Florida, Jacksonville, Florida. Electronic address: Harnois.Denise@mayo.edu.
Abstract
BACKGROUND: Graft-versus-host disease (GVHD) is a rare, serious, fatal disease that occurs after orthotopic liver transplantation (OLT). CASE REPORT: We treated a 60-year-old man who underwent OLT owing to familial amyloidosis. The patient developed fever on postoperative day 16. The fever was persistent and did not respond to antibiotic therapy. Cultures and radiologic studies were done and excluded infection as a potential cause. On postoperative day 26, a skin rash appeared on his chest, accompanied by diarrhea and persistent fever. The rash spread all over the trunk, neck, and arms, but spared the palms of his hands and soles of his feet. In the meantime, his blood cell count revealed pancytopenia. Skin biopsy was done and showed interface lymphocytic infiltrate that are largely centered on the dermal-epidermal junction, is consistent with GVHD (this pattern of rash distribution is unique and different from the rash of GVHD after hematopoietic stem cell transplant, which is confined to palms of the hands and soles of the feet; Fig 1). The diagnosis was confirmed by colonoscopy and multiple forceps biopsies, which revealed extensive crypt loss. After hematology consultation, the patient was treated by withdrawal of all immunosuppressive therapy coupled with abatacept infusion. Abatacept is a chimeric protein that inhibits T-lymphocytes and is approved by the US Food and Drug Administration for the treatment of rheumatoid arthritis. Interestingly, after second dose of abatacept the patient showed marked clinical and laboratory improvement. The patient was discharged after 47 days in a stable condition. CONCLUSION: Because of the lack of a consensus for treatment of these patients, we report our experience with a male patient who had post-OLT GVHD and showed a marked improvement in response to abatacept.
BACKGROUND:Graft-versus-host disease (GVHD) is a rare, serious, fatal disease that occurs after orthotopic liver transplantation (OLT). CASE REPORT: We treated a 60-year-old man who underwent OLT owing to familial amyloidosis. The patient developed fever on postoperative day 16. The fever was persistent and did not respond to antibiotic therapy. Cultures and radiologic studies were done and excluded infection as a potential cause. On postoperative day 26, a skin rash appeared on his chest, accompanied by diarrhea and persistent fever. The rash spread all over the trunk, neck, and arms, but spared the palms of his hands and soles of his feet. In the meantime, his blood cell count revealed pancytopenia. Skin biopsy was done and showed interface lymphocytic infiltrate that are largely centered on the dermal-epidermal junction, is consistent with GVHD (this pattern of rash distribution is unique and different from the rash of GVHD after hematopoietic stem cell transplant, which is confined to palms of the hands and soles of the feet; Fig 1). The diagnosis was confirmed by colonoscopy and multiple forceps biopsies, which revealed extensive crypt loss. After hematology consultation, the patient was treated by withdrawal of all immunosuppressive therapy coupled with abatacept infusion. Abatacept is a chimeric protein that inhibits T-lymphocytes and is approved by the US Food and Drug Administration for the treatment of rheumatoid arthritis. Interestingly, after second dose of abatacept the patient showed marked clinical and laboratory improvement. The patient was discharged after 47 days in a stable condition. CONCLUSION: Because of the lack of a consensus for treatment of these patients, we report our experience with a male patient who had post-OLT GVHD and showed a marked improvement in response to abatacept.
Authors: Grace Y Kim; Leah A Schmelkin; Mark D P Davis; Rokea A El-Azhary; Ann M Farrell; Alexander Meves; Julia S Lehman Journal: J Am Acad Dermatol Date: 2017-12-27 Impact factor: 11.527