Published online Aug 18, 2022. doi: 10.5500/wjt.v12.i8.268
Peer-review started: January 7, 2022
First decision: March 9, 2022
Revised: March 24, 2022
Accepted: August 5, 2022
Article in press: August 5, 2022
Published online: August 18, 2022
Processing time: 223 Days and 0.9 Hours
Post-transplant lymphoproliferative disorder (PTLD) is one of the most common post-transplant malignancies within the gastrointestinal (GI) tract. PTLD is a lymphoma variant which can manifest in patients having solid organ transplantation (SOT) or hematopoietic stem cell transplantation (HSCT).
The current understanding of GI manifestations of PTLD including timing to development, risk factors for development, and treatment is limited by small sample size. Previous studies have noted a propensity for the GI tract to develop PTLD; therefore, more information regarding when it may develop, how it manifests, and treatments are needed especially as transplantation becomes more prevalent.
To identify the timing and clinical presentation of GI-PTLD, risk factors for its development, and treatment.
We performed a systematic review after an extensive literature search.
The timing of GI-PTLD is variable but on average develops 4-5 years following SOT and may occur within 1 year after HSCT. Presentation may be insidious including nonspecific abdominal discomfort to fulminant hemorrhage, perforation, or obstruction. GI-PTLD is most likely to develop in the small intestine and stomach. Transplant type, level of induction and maintenance immunosuppression, Epstein-Barr virus-status among other risk factors increase the likelihood one may develop PTLD. PTLD is aggressive and mortality improves with early treatment which is dependent on extent of disease, and morphological subtype. The most important step of therapy is reduction of immunosuppression (RIS) which usually is effective.
The presentation, imaging, and direct appearance of GI-PTLD is highly variable making clinical suspicion key for diagnosis. Early detection is key for prognosis; therefore, consideration of risk factors is essential. Treatment is dependent on several factors and may include RIS, rituximab, chemotherapy, surgery, or a combination of these interventions. Initial treatment is intuitive and technically easy; however, RIS can be associated with acute graft rejections.
This study suggests ascertainment of risk factors is crucial for increasing clinical suspicion when assessing patients who may have GI-PTLD. The clinical and radiological presentation of GI-PTLD is highly variable; therefore, a high index of suspicion for GI-PTLD must be maintained so that early endoscopic diagnosis may allow for targeted treatment. Future prospective studies are needed to better elucidate incidence rates of GI-PTLD and the role of endoscopy in treatment.