553 Clinical characteristics of acute graft-versus-host disease in small bowel and multi-visceral transplant recipients

Abstract

Acute graft-versus-host disease (aGVHD) following stem cell transplantation is characterized by rash, liver dysfunction, and diarrhea. Although aGVHD is uncommon following solid organ transplantation, we find higher rates of aGVHD after intestinal (ITx) and multi-visceral transplantation (MVT). In these cases we find a unique presentation as the donor liver (if present) and intestine are spared, leaving skin as the main target. Between 2013 and 2019, we diagnosed 17 cases of aGVHD following ITx (n=9) and MVT including both intestine and liver (n=8). Median time to onset of aGVHD was 37 days. Erythema on the trunk, palms, or both was the most common presenting skin finding (15/17 patients). All patients had skin biopsies at the time of diagnosis. Patients presented with grade 1 (7/17), grade 2 (8/17), grade 3 (1/17), and grade 4 (1/17) aGVHD. Peripheral blood chimerism studies obtained in 11 patients revealed donor CD3+ lymphocytes in 5 (45%). Two patients had passenger lymphocyte syndrome, and partial donor stem cell engraftment was diagnosed in a third patient. Native GI tract involvement (usually rectosigmoid), was found in 7/17 patients; other sites included bone marrow, and native liver. In all, 6/17 patients had no evidence of extra-cutaneous aGVHD. Treatment strategies included systemic and topical corticosteroids, anti-thymocyte globulin, extracorporeal photopheresis, and/or JAK inhibitors. Complications of immunosuppression including opportunistic infections (aspergillosis and toxoplasmosis) and post-transplant lymphoproliferative disorder occurred in 7/17 patients. In summary, rash is typically the first, and may be the only, manifestation of aGVHD following luminal organ transplants. Diagnosis may be aided by time of onset of cutaneous manifestations, extra-cutaneous signs, and peripheral blood chimerism.