Introduction: Graft-versus-host disease (GVHD) is known as a rare but life-threatening complication after liver transplantation (LT), and reports of successful treatment for GVHD after LT were extremely rare. In this presentation, we describe a case of acute GVHD following deceased donor liver transplantation (DDLT) who survived with the withdrawal of immunosuppressive (IS) therapy, an aggressive supportive treatment and bowel resection.
Case Presentation: The patient was a 35-year-old man who had undergone DDLT for fulminant hepatitis B. The HLA phenotype of the recipient was A2,33; B44,46; DR8. The HLA phenotype of the donor was A2,11;B71,46;DR8,14. His postoperative course was uneventful with the dual IS therapy. On postoperative day 82, the patient was emergently admitted for melena and voluminous diarrhea with generalized erythema. The results of skin biopsy, duodenal biopsy, and T-cell chimerism analysis led to a diagnosis of acute GVHD. On chimerism analysis, 22% of peripheral blood T-cells were assumed to be of donor origin. According to the previous successful report of acute GVHD after liver transplantation, we decided to discontinue the IS agents, which resulted in the complete depletion of donor-derived T-cells within three weeks. Meanwhile, acute cellular rejection of the liver allograft was observed 14 days after the discontinuation of IS, at which moment the dual IS treatment was resumed. While the skin rash got cured rapidly, the severe melena and diarrhea (3000-8000ml/day) demanding massive amount of blood products lasted for 3 months. However, the protein-losing enteropathy was exacerbated by oral intake. For this reason, the patient was discharged on POD 260 with home parenteral nutrition. On POD 389, he developed to uncontrollable severe fever with bowel obstructive symptoms. We performed emergency surgery on POD 391. The segmentally distended ileum and circumferential bowel wall thickening were found at laparotomy. Finally, we resected 250cm of small intestine. Histopathological diagnosis confirmed a neutrophilic infiltrate and apoptotic bodies at the stenotic site. The postoperative course was uneventful and oral intake was rapidly become possible with no dietary restrictions. Three years have already passed since DDLT, the patient was fully return to his normal lives.
Conclusion: Based on the literature review, the treatment strategy is controversial from the increment to the cessation of IS. The complete withdrawal of IS followed by a gradual resuming at the time of acute rejection of liver allograft in the present case may be a reasonable treatment option for GVHD after LT. Furthermore, intestinal resection for residual intestinal symptoms was key elements of the successful treatment. This is the world's first case of GVHD successfully cured by intestinal resection performed after withdrawal of immunosuppressant. Moreover, this is the first life-saving case from acute GVHD occurring after liver transplantation in Japan.