Rare presentation of post-transplant lymphoproliferative disorder isolated to gastroesophageal junction

Abstract

Post transplant lymphoproliferative disorder (PTLD) represents a life threatening disorder occurring after transplantation, ranging from a polyclonal mononucleosis like illness to a monomorphic high grade neoplasm with cytologic and histopathologic evidence indicative of transformation to lymphoma. PTLD of diffuse large B-cell lymphoma (DLBCL) subtype, isolated to the esophagus is a rare diagnosis. We describe the first case of an immunocompromised adult patient diagnosed with DLBCL-PTLD limited to his esophagus without an associated mass or locoregional lymphadenopathy on imaging since the institution of the revised Cheson criteria, which includes positron emission tomography-computed tomography as the standard staging modality. Even more unique to our case was the suggestion of underlying cytomegalovirus (CMV) gastritis leading to a hypothesis about a less well understood relationship between CMV and Epstein Barr virus (EBV). In the post transplant setting, immunocompromised state, or EBV positive state, upper gastrointestinal symptoms should prompt investigation with an upper endoscopy (EGD). Additionally, specific to our case, the fact that the patients’ presentation was suspicious for CMV gastritis raises the possibility that the CMV infection predated his PTLD increasing his risk of acquiring PTLD. This reemphasizes the importance and diagnostic utility of early screening with EGD in patients after transplantation.

Keywords: B-cell lymphoma, Esophageal, Lymphoproliferative, Post-transplant, Epstein barr virus

Core tip: Post transplant lymphoproliferative disorder (PTLD) are a heterogeneous group of lymphoid proliferations associated with immunosuppression following solid organ transplantation (SOT) or allogeneic hematopoietic stem cell transplantation (HSCT). PTLD associated with B-cell esophageal lymphoma is a rare diagnosis. Our patient was identified to have isolated PTLD at the gastroesophageal junction, which has not been reported in the modern era of positron emission tomography-computer tomography. Previous reported cases describe esophageal lymphoma associated with symptoms of dysphagia, odynophagia or esophogastroduodenoscopy (EGD) findings that revealed an associated mass or locoregional lymphadenopathy. However, given the variation in presentation in post-transplant and immunocompromised patients, gastrointestinal symptoms should prompt further investigation with an EGD.