Characteristics and Outcome of HIV-Associated Primary Effusion Lymphoma (PEL) As Observed in the German HIV-Related Lymphoma Cohort Study
Authors: Hentrich Marcus , Schommers Philipp , Mülller Markus , Wyen Christoph , Thomssen Henrike , Wolf Timo , Wasmuth Jan-Christian , Bogner Johannes , Hoffmann Christian 
Published: October 2019

Abstract
Introduction: Human immunodeficiency virus (HIV)-associated primary effusion lymphoma (PEL) is a rare and aggressive subtype of B-cell Non-Hodgkin lymphomas (NHL). PEL usually arises in body cavities such as the peritoneum, the pleural space, and pericardium, but may also present as extracavitary lesions without effusion. Definitive diagnosis hinges on detection of viral infection by human herpes virus 8 (HHV-8) in the neoplastic cells. Optimal treatment for HIV-PEL has not been defined. The aim of the present study was to analyze clinical characteristics, treatment and outcome of pts with HIV-PEL who were included in the German HIV-related lymphoma cohort study.
Methods: The German HIV-related lymphoma cohort study is a prospective multicenter study that includes adult HIV-1 infected pts with biopsy or cytologically proven HIV-related lymphoma. Patients were diagnosed at 32 participating centers in Germany and Austria since January 2005. Data on HIV-infection and lymphoma characteristics, treatments and outcomes were recorded. The present analysis focuses on pts with PEL.
Results: Twelve out of 437 pts (2.7%) with HIV-NHL were diagnosed with PEL. All pts were male, and the median age at PEL diagnosis was 51.8 yrs (range, 35-64). 11 of 12 pts (92%) had stage IV disease and B-symptoms were present in 8 pts (67%). Five pts were diagnosed with a solid variant consisting of nodal and gastrointestinal (n=2), myocardial followed by gastrointestinal (n=1), pulmonary and lymph node (n=1), and lymph node followed by cutaneous involvement (n=1). The pleural space, pericardium, and peritoneum were affected in 6, 1, and 4 cases, respectively, with more than one body cavity involved in 4 pts. The median CD4-lymphocyte count at lymphoma diagnosis was 174/µl (range, 48-492), and 8 pts (67%) had an AIDS-defining illness prior to diagnosis of PEL. All but 3 pts (75%) were on combination antiretroviral treatment (cART) at time of PEL diagnosis, and HIV-RNA was below the limit of detection in 9 pts (67%). First line chemotherapy (CT) consisted of CHO(E)P-like regimens in 8 pts, liposomal doxorubicin for concurrent Kaposi sarcoma in 2 pts, and rituximab/bortezomib/liposomal doxorubcin in 1 case. One patient only received intrapleural mitoxantrone for pleural effusion. First salvage CT consisted of EPOCH (n=3) or DHAP with and without high dose chemotherapy (n=3). Further salvage treatments included the dose intensive GMALL B-ALL/B-NHL protocol, brentuximab vedotin, EPOCH, and lenalidomide, given to one patient each. After a median follow-up of 12 months for the entire study population, 2 pts (17%) were alive and in continuous complete remission. The 2-year-overall survival was 16.7%. Patients died of refractory lymphoma (n=5), neutropenic sepsis (n=1), pneumonia/non-neutropenic sepsis (n=2), hyperinflammatory syndrome (n=1), and in one case the cause of death remained unclear.
Conclusions: The overall survival of patients with HIV-PEL remains poor, although long term survival may be achieved in selected cases. International collaborative studies may help to define the optimal treatment approach.

Disclosures
No relevant conflicts of interest to declare.