Primary effusion lymphoma occurring in the setting of transplanted patients: a systematic review of a rare, life-threatening post-transplantation occurrence

BMC Cancer

Alessandra Soriano¹  Giovanni Martino²  Maria Chiara Bassi³  Stefano Ascani⁴  Cecilia Caprera⁴  Linda Ricci⁴  Matteo Corsi⁴  Francesca Sanguedolce⁵  Magda Zanelli⁶  Stefano Ricci⁶  Andrea Palicelli⁶  Giacomo Santandrea⁶  Maurizio Zizzo⁷ 

[1] Gastroenterology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123, Reggio Emilia, Italy;Hematology Unit, CREO, Azienda Ospedaliera di Perugia, University of Perugia, 06121, Perugia, Italy;Medical Library, Azienda USL-IRCCS di Reggio Emilia, 42123, Reggio Emilia, Italy;Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100, Terni, Italy;Pathology Unit, Azienda Ospedaliera Universitaria “Ospedali Riuniti” di Foggia, 71122, Foggia, Italy;Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123, Reggio Emilia, Italy;Surgical Oncology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123, Reggio Emilia, Italy;Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, 41121, Modena, Italy;

关键词: Lymphoma;    Effusion;    Epstein-Barr virus;    Human Herpesvirus 8;    Transplantation;   

DOI  :  10.1186/s12885-021-08215-7

来源: Springer

【 摘 要 】

BackgroundPrimary effusion lymphoma is a rare, aggressive large B-cell lymphoma strictly linked to infection by Human Herpes virus 8/Kaposi sarcoma-associated herpes virus. In its classic form, it is characterized by body cavities neoplastic effusions without detectable tumor masses. It often occurs in immunocompromised patients, such as HIV-positive individuals. Primary effusion lymphoma may affect HIV-negative elderly patients from Human Herpes virus 8 endemic regions. So far, rare cases have been reported in transplanted patients. The purpose of our systematic review is to improve our understanding of this type of aggressive lymphoma in the setting of transplantation, focusing on epidemiology, clinical presentation, pathological features, differential diagnosis, treatment and outcome. The role of assessing the viral serological status in donors and recipients is also discussed.MethodsWe performed a systematic review adhering to the PRISMA guidelines. The literature search was conducted on PubMed/MEDLINE, Web of Science, Scopus, EMBASE and Cochrane Library, using the search terms “primary effusion lymphoma” and “post-transplant”.ResultsOur search identified 13 cases of post-transplant primary effusion lymphoma, predominantly in solid organ transplant recipients (6 kidney, 3 heart, 2 liver and 1 intestine), with only one case after allogenic bone marrow transplantation. Long-term immunosuppression is important in post-transplant primary effusion lymphoma commonly developing several years after transplantation. Kaposi Sarcoma occurred in association with lymphoma in 4 cases of solid organ recipients. The lymphoma showed the classical presentation with body cavity effusions in absence of tumor masses in 10 cases; 2 cases presented as solid masses, lacking effusions and one case as effusions associated with multiple organ involvement. Primary effusion lymphoma occurring in the setting of transplantation was more often Epstein Barr-virus negative. The prognosis was poor. In addition to chemotherapy, reduction of immunosuppressive treatment, was generally attempted.ConclusionsPrimary effusion lymphoma is a rare, but often fatal post-transplant complication. Its rarity and the difficulty in achieving the diagnosis may lead to miss this complication. Clinicians should suspect primary effusion lymphoma in transplanted patients, presenting generally with unexplained body cavity effusions, although rare cases with solid masses are described.

【 授权许可】

CC BY