title: Classic and extracavitary primary effusion lymphoma in 51 HIV-infected patients from a single institution.
abstract: Human immunodeficiency virus (HIV)-associated primary effusion lymphoma (PEL) is a rare B-cell non-Hodgkin lymphoma with poor prognosis. Lymphoma cells are always infected with human herpesvirus-8 (HHV-8) and in most cases coinfected with Epstein-Barr virus. In classic presentation, PEL is characterized by body cavity effusions with or without mass lesions. A variant with only extracavitary localization has also been described. We report on a large single-center series of patients with PEL in the era of combined antiretroviral therapy (cART). The main objective was to compare the characteristics and the outcome of patients with classic (n = 34) and extracavitary (n = 17) variant PEL. At PEL diagnosis, no major difference was observed between the two groups in terms of demographic and HIV characteristics. Extracavitary localizations were exclusively nodal in six patients and involved various organs in 11 patients. Another HHV-8-associated disease was observed in 31 patients, Kaposi sarcoma in 25, and multicentric Castleman disease in 18 patients, without difference between the two groups. Thirty-two patients were treated with CHOP associated with high-dose methotrexate, 13 were treated with CHOP-derived regimen alone, and six patients received low-dose/no chemotherapy. Complete remission was achieved in 21 (62%) and seven (41%) patients of the classic and extracavitary groups, respectively. The median overall survival (OS) was 10.2 months. Despite a higher disease-free survival in the extracavitary group, there was no difference in OS between the two variants. Based on this series, characteristics of classic and extracavitary variants were very close. Although prognosis of PEL remains very severe in cART era, the median survival compares favorably with earlier series.
authors.affiliation: Department of Clinical Immunology, Saint-Louis Hospital, AP-HP, Paris, France.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Guillet
authors.affiliation: Department of Clinical Immunology, Saint-Louis Hospital, AP-HP, Paris, France.|EA3518, University Paris Diderot, Paris, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Gérard
authors.affiliation: EA3518, University Paris Diderot, Paris, France.|Department of Pathology, Saint-Louis Hospital, AP-HP, Paris, France.
authors.forename: Véronique
authors.initials: V
authors.lastname: Meignin
authors.affiliation: EA3518, University Paris Diderot, Paris, France.|Virology Laboratory, Saint-Louis Hospital, AP-HP, Paris, France.
authors.forename: Felix
authors.initials: F
authors.lastname: Agbalika
authors.affiliation: Hematology Laboratory, Saint-Louis Hospital, AP-HP, Paris, France.
authors.forename: Wendy
authors.initials: W
authors.lastname: Cuccini
authors.affiliation: Department of Infectious Diseases, Saint-Louis Hospital, AP-HP, Paris, France.
authors.forename: Blandine
authors.initials: B
authors.lastname: Denis
authors.affiliation: Department of Infectious Diseases, Pitié-Salpêtrière Hospital, AP-HP, Paris, France.
authors.forename: Christine
authors.initials: C
authors.lastname: Katlama
authors.affiliation: Department of Clinical Immunology, Saint-Louis Hospital, AP-HP, Paris, France.|EA3518, University Paris Diderot, Paris, France.
authors.forename: Lionel
authors.initials: L
authors.lastname: Galicier
authors.affiliation: Department of Clinical Immunology, Saint-Louis Hospital, AP-HP, Paris, France.|EA3518, University Paris Diderot, Paris, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Oksenhendler
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D018572:Disease-Free Survival; D005260:Female; D015658:HIV Infections / Q000188:drug therapy* / Q000235:genetics / Q000401:mortality / Q000821:virology; D006801:Humans; D053208:Kaplan-Meier Estimate; D007621:Karyotyping; D016483:Lymphoma, AIDS-Related / Q000188:drug therapy* / Q000235:genetics / Q000401:mortality / Q000821:virology; D054685:Lymphoma, Primary Effusion / Q000188:drug therapy* / Q000235:genetics / Q000401:mortality / Q000821:virology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D016896:Treatment Outcome