Extracavitary primary effusion lymphoma in a post‐transplantation patient

Abstract

A 42-year-old human immunodeficiency virus (HIV)-negative man underwent small bowel transplantation in March 2005. He had a history of allergic rhinitis, asthma, neuropathies and hypereosinophilia as a result of Churg–Strauss syndrome (CSS), diagnosed in January 2004. Despite systemic corticosteroids and cyclophosphamide, in May 2004 he required emergency surgery for multiple small intestine perforations, related to small vessels angiitis and consistent with CSS. After massive small bowel resection, small bowel transplantation was the only life-saving option. Prior to transplantation, he had no detectable viraemia for either Epstein–Barr virus (EBV) or human herpesvirus 8 (HHV8) by polymerase chain reaction. Immunosuppressive treatment post-transplantation included tacrolimus and monoclonal anti-CD52 antibody (alemtuzumab). Seven months later, he required hospitalisation for hyperpyrexia, weight loss and epigastric pain. Upper gastrointestinal endoscopy revealed multiple gastric and duodenal ulcerated polyps, maximum 3 cm in diameter. A polyps biopsy showed a diffuse infiltrate of large cells, with eccentric nuclei containing prominent nucleoli consistent with plasmablasts. The cells expressed plasma cells markers (CD138, CD38), CD30, epithelial membrane antigen and lambda light chain markers and lacked B-cell (CD20, CD79a, PAX5) and T-cell (CD3, CD5) markers. The proliferative index (Ki-67) was about 70%. On immunohistochemistry, the cells were diffusely positive for HHV8 latent nuclear antigen 1 (left). EBV was identified by in situ hybridisation for EBV-encoded RNA in the large cells (right). A monomorphic post-transplantation lymphoproliferative disorder consistent with primary effusion lymphoma (PEL), extracavitary variant was diagnosed. At the time of diagnosis, high EBV and HHV8 viral loads were detected in peripheral blood. No other site of disease was present on a positron emission tomography scan. Bone marrow trephine biopsy was negative. The patient deteriorated rapidly and died 1 month later. PEL is a rare aggressive B-cell non-Hodgkin lymphoma, usually arising in a body cavity and presenting with an effusion, without any identifiable tumour mass. Rare cases, presenting as a solid mass without effusion, have been termed extracavitary PEL. This lymphoma occurs in HIV-positive patients and rarely in HIV-negative elderly patients. It is seldom reported in immunosuppressed patients, following solid organ transplantation. PEL is causally linked to HHV8 with EBV coinfection, especially in HIV-positive patients. PEL, in its effusion-based form, has been reported in at least 13 solidorgan recipients (kidney and heart), frequently with a high HHV8 viral load. The present case is a rare example of extracavitary PEL, occurring in a small bowel transplant recipient.