Therapeutic Challenges in Post-Transplant Lymphoproliferative Disorder of the Esophagus with Central Nerve System Involvement Complicated by an Esophagotracheal Fistula: A Case Report

Abstract

.1 Background: Post-transplant lymphoproliferative disorder (PTLD) is a known complication following solid organ or hematopoietic stem cell transplantation. It’s a heterogeneous group of lymphoid and/or plasmocytic proliferations as a result of immunosuppression and is frequently associated with Epstein-Barr Virus. The clinical presentation is nonspecific and highly variable depending on the localization. We discuss the therapeutic challenges in this rare case of a male suffering PTLD with esophageal involvement complicated by an esophagotracheal fistula. 1.2 Case Report: A 55-year-old man under immunosuppression after kidney transplantation, presented with progressive cough and fever after elective upper endoscopy. Following the diagnosis of diffuse large B cell lymphoma-type PTLD with esophagotracheal fistula. The reduction of immunosuppression was unable to control the progression of disease with involvement of the CNS. Endoscopic repair of the fistula was unsuccessful. The need for surgical repair of the fistula delayed therapy with rituximab by one month. Despite brain radiation therapy, the patient passed away 3.5 months after diagnosis. 1.3 Conclusion: In patients with esophagotracheal fistula both aspiration pneumonia and treatment of the fistula can delay effective treatment of the underlying malignant disease. In patients with PTLD reduction in immunosuppression is the primary treatment strategy, followed by monotherapy with the monoclonal antibody rituximab in the case of incomplete response. Newer therapeutic approaches prefer the sequential treatment with rituximab followed by chemotherapy in case of incomplete response, or consolidation therapy with rituximab in case of complete response. Radiotherapy is recommended in case of CNS involvement.