Gastrointestinal manifestations, risk factors, and management in patients with post-transplant lymphoproliferative disorder: A systematic review

Abstract

BACKGROUND Patients with a history of solid organ transplantation (SOT) or hematopoietic stem cell transplantation (HSCT) are at an increased risk of developing post-transplant lymphoproliferative disorder (PTLD). The gastrointestinal (GI) tract is commonly affected as it has an abundance of B and T cells. AIM To determine typical GI-manifestations, risk factors for developing PTLD, and management. METHODS Major databases were searched until November 2021. RESULTS Non-case report studies that described GI manifestations of PTLD, risk factors for developing PTLD, and management of PTLD were included. Nine articles written within the last 20 years were included in the review. All articles found that patients with a history of SOT, regardless of transplanted organ, have a propensity to develop GI-PTLD. CONCLUSION GI tract manifestations may be nonspecific; therefore, consideration of risk factors is crucial for identifying GI-PTLD. Like other lymphoma variants, PTLD is very aggressive making early diagnosis key to prognosis. Initial treatment is reduction of immunosuppression which is effective in more than 50% of cases; however, additional therapy including rituximab, chemotherapy, and surgery may also be required.

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“…Among SOT recipients, the incidence of PTLD ranges from 2% to 30% depending upon the organ type; liver, small bowel, and multivisceral transplants carrying the higher risk (36). GI involvement is frequent due to the large number of resident lymphocytes (37). Clinical manifestations of GI PTLD are varied and can be vague: fever, malaise, abdominal fullness, nausea, vomiting, diarrhea, occult gastrointestinal bleeding, frank hematochezia, intestinal obstruction, and intestinal perforation (37).…”

“…GI involvement is frequent due to the large number of resident lymphocytes (37). Clinical manifestations of GI PTLD are varied and can be vague: fever, malaise, abdominal fullness, nausea, vomiting, diarrhea, occult gastrointestinal bleeding, frank hematochezia, intestinal obstruction, and intestinal perforation (37).…”

“…First line management of PTLD is reduction of immunosuppression (36). Most experts recommend a 50% reduction in calcineurin inhibitor with discontinuation of azathioprine or MMF (37). GI disease typically also requires treatment with rituximab and/or chemotherapy depending on the histology.…”

“…There are trials that support the use of low-dose cyclophosphamide and prednisone to treat rituximab-refractory PTLD (39). In the case of classical Hodgkin lymphoma, reduction of immunosuppression is not validated and alternative chemotherapeutic regimens may be recommended (36,37). Surgical resection may be required in the case of GI hemorrhage or perforation (36).…”