Post-transplant lymphoproliferative disorder of the ileum diagnosed by double-balloon enteroscopy

Post-transplant lymphoproliferative disorder (PTLD) is a common life-threatening complication after solid-organ transplantation that is mostly related to Epstein–Barr virus (EBV) infection [1]. PTLD occurs in 6.2 % of lung transplants, 5.2 % of kidney and pancreas transplants, 2 % of heart transplants, and 1.4 % of liver transplants [2]. Although around 15 % of patients present as an emergency with intestinal perforation [3], there are only a few reports of cases in which PTLD lesions in the small intestine have been observed endoscopically. We report a case of PTLD involving the ileum that occurred 11 months after heart transplantation and was detected during double-balloon enteroscopy (DBE).

A 49-year-old man who had undergone a heart transplant 11 months previously and was receiving 10 mg tacrolimus, 2.5 mg everolimus, and 5 mg prednisolone per day was admitted to our hospital because of high fever and passage of a tarry stool. Esophagogastroduodenoscopy (EGD) showed an ulcer in the posterior wall of the stomach ([Fig. 1 a]), which was later shown on pathological examination to contain a nonspecific inflammatory infiltrate. Further examination with DBE revealed multiple ulcers in the ileum ([Fig. 1 b]), which were later shown to be positive on fluorodeoxyglucose-positron emission tomography (FDG-PET).

Fig. 1 Endoscopic images showing: a an ulcer seen during esophagogastroduodenoscopy on the posterior wall of the stomach (later histological diagnosis was nonspecific); b multiple ulcers detected in the ileum by double-balloon enteroscopy (DBE).

Treatment was started with fasting and total parenteral nutrition, but his condition remained unchanged. After a month, he complained of sudden severe abdominal pain, which was caused by perforation of the ileal ulcers, and he underwent emergent segmental ileal resection. Pathological examination of the resection specimen revealed a diagnosis of diffuse large B-cell lymphoma caused by PTLD ([Fig. 2 a]) and staining for EBV-encoded RNA (EBER) was positive ([Fig. 2 b]). He therefore received treatment with rituximab, and a subsequent FDG-PET scan showed that the FDG-avid stomach lesion had disappeared.

Fig. 2 Pathological appearance of the resected ileum showing: a diffuse large B-cell lymphoma caused by post-transplant lymphoproliferative disorder (PTLD); b positivity (brown staining) for EBV-encoded RNA (EBER).

There have been few reported cases of PTLD-related ileal lesions being diagnosed by endoscopy. Nevertheless, the possibility of PTLD must be considered when multiple ileal ulcers are observed after organ transplantation.

Endoscopy_UCTN_Code_CCL_1AC_2AC

• References

• 1 Tanner JE, Alfieri C. The Epstein–Barr virus and post-transplant lymphoproliferative disease: interplay of immunosuppression, EBV, and the immune system in disease pathogenesis. Transpl Infect Dis 2001; 3: 60-69
• 2 Walker RC, Paya CV, Marshall WF et al. Pretransplantation seronegative Epstein–Barr virus status is the primary risk factor for posttransplantation lymphoproliferative disorder in adult heart, lung, and other solid organ transplantations. J Heart Lung Transplant 1995; 14: 214-221
• 3 Kahan BD, Ponticelli C, Montagnino G eds. Principles and practice of renal transplantation. London: Martin Dunitz; 2000