A Comprehensive Clinicopathologic and Molecular Study of 19 Primary Effusion Lymphomas in HIV-infected Patients. (22nd March 2022)

• Record Type: Journal Article
• Authors: Calvani, Julien; Gérard, Laurence; Fadlallah, Jehane; Poullot, Elsa; Galicier, Lionel; Robe, Cyrielle; Garzaro, Margaux; Bertinchamp, Remi; Boutboul, David; Cuccuini, Wendy; Cayuela, Jean-Michel; Gaulard, Philippe; Oksenhendler, Éric; Meignin, Véronique
• Is Part Of: American journal of surgical pathology. Volume 46:Number 3(2022)
• Page Start: 353
• Page End: 362
• Publication Date: 2022-03-22
• Subjects: primary effusion lymphoma -- PD-L1 -- PD-1 -- EBV -- HIV -- KSHV/HHV-8; Pathology, Surgical -- Periodicals; 617.0705
• DOI: 10.1097/PAS.0000000000001813
• Languages: English
• ISSNs: 0147-5185

Abstract

Abstract : Primary effusion lymphoma (PEL) is associated with human herpesvirus 8 and frequently with Epstein-Barr virus (EBV). We report here a single-center series of 19 human immunodeficiency virus–associated PELs, including 14 EBV + and 5 EBV − PELs. The objectives were to describe the clinicopathologic features of PELs, with a focus on programmed cell death protein 1 (PD-1)/programmed death-ligand 1 (PD-L1) expression, to search for genetic alterations by targeted deep sequencing analysis, and to compare the features between EBV + and EBV − cases. All the patients were male, and the median age at diagnosis was 47 years old (interquartile range: 40 to 56 y). Reflecting the terminal B-cell differentiation, immunophenotypic profiles showed low expression levels of B-cell markers, including CD19 (0/19), CD20 (1/19), CD79a (0/19), PAX5 (1/19), BOB1 (3/19), and OCT2 (4/19), contrasting with a common expression of CD38 (10/19), CD138 (7/19), and IRF4/MUM1 (18/19). We observed a frequent aberrant expression of T-cell markers, especially CD3 (10/19), and less frequently CD2 (2/19), CD4 (3/19), CD5 (1/19), and CD8 (0/19). Only 2 cases were PD-L1 positive on tumor cells and none PD-1 positive. With respect to immune cells, 3 samples tested positive for PD-L1 and 5 for PD-1. Our 36-gene lymphopanel revealed 7 distinct variants in 5/10 PELs, with either a single or 2 mutations per sample: B2M (n=2), CD58 (n=1), EP300 (n=1), TNFAIP3 (n=1), ARID1A (n=1), and TP53 (n=1). Finally, we did not observe any major clinical, pathologic, or immunohistochemical differences between EBV + and EBV − PELs and the outcome was similar (2-y overall survival probability of 61.9% [95% confidence interval, 31.2-82.1] vs. 60.0% [95% confidence interval, 12.6-88.2], respectively, P =0.62).