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.

Posttransplant Lymphoproliferative Disorder of the Small Bowel as an Unexpected Cause of Iron Deficiency Anemia Decades after Heart Transplantation

“…The median time to presentation after transplant was 2.9 years, and most demonstrated ulceration and/or perforation on endoscopy 10. There are very few additional reports of isolated PTLD of the GI tract in the adult transplant population 6,11…”

“…Studies demonstrate that up to half of patients with PTLD have evidence of disease in the small bowel, colon, or mesenteric lymph nodes. 5 , 6 However, isolated PTLD of the GI tract is rare. As PTLD is more common among pediatric patients, isolated GI PTLD is best described in pediatric literature.…”

“… 10 There are very few additional reports of isolated PTLD of the GI tract in the adult transplant population. 6 , 11 …”

Posttransplant Lymphoproliferative Disorder: Otolaryngological Manifestations and Management

“…50 Esophageal PTLD limited to the esophagus is rare, and symptoms are usually nonspecific (dysphagia, odynophagia, retrosternal burning, etc). 51,52 A more common posttransplant manifestation is eosinophilic esophagitis, which is regarded as a chronic immune/antigen-mediated disease with biopsy crucial to confirming diagnosis. 53 Posttransplant eosinophilic esophagitis is more common in children.…”

Post-transplant lymphoproliferative disorder as a cause of oesophageal ulceration and stricture: case report and literature review

“…Development of PTLD is commonly a result of uncontrolled B cell proliferation in the context of Epstein-Barr virus (EBV) infection. 1 The clinical presentation of PTLD is variable, with some patients being completely asymptomatic and others presenting in critical condition with multiorgan involvement. Common organ involvement in PTLD includes lymph nodes, the central nervous system and gastrointestinal system.…”