Viral, immunologic, and clinical features of primary effusion lymphoma
Autor: | Wyndham H. Wilson, Manisha Bhutani, Armando C. Filie, Thomas S. Uldrick, Robert Yarchoan, Denise Whitby, Seth M. Steinberg, Mark N. Polizzotto, Karen Aleman, Richard F. Little, Wendell Miley, Stefania Pittaluga, Priscila H. Goncalves, Kathryn Lurain, Vickie Marshall, Elaine S. Jaffe, Ramya Ramaswami, Kathleen M. Wyvill |
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Rok vydání: | 2018 |
Předmět: |
Adult
Male medicine.medical_specialty Vincristine Herpesvirus 4 Human viruses Immunology Biochemistry Gastroenterology Young Adult immune system diseases Internal medicine hemic and lymphatic diseases Lymphoma Primary Effusion medicine Humans EPOCH (chemotherapy) Sarcoma Kaposi Etoposide Survival analysis Aged Lymphoid Neoplasia business.industry Interleukin-6 Castleman Disease Hazard ratio virus diseases Cell Biology Hematology biochemical phenomena metabolism and nutrition Middle Aged medicine.disease Prognosis Survival Analysis Lymphoma Interleukin-10 Herpesvirus 8 Human Cytokines Female Primary effusion lymphoma Lymphoma Large B-Cell Diffuse business Diffuse large B-cell lymphoma medicine.drug |
Zdroj: | Blood. 133(16) |
ISSN: | 1528-0020 |
Popis: | Primary effusion lymphoma (PEL) is an aggressive HIV-associated lymphoma with a relatively poor prognosis in the era of effective HIV therapy. Kaposi sarcoma herpesvirus (KSHV) is the etiologic agent, and ∼80% of tumors are coinfected with Epstein-Barr virus (EBV). A better understanding of how KSHV-related immune dysregulation contributes to the natural history of PEL will improve outcomes. Twenty patients with PEL diagnosed between 2000 and 2013, including 19 treated with modified infusional etoposide, vincristine, and doxorubicin with cyclophosphamide and prednisone (EPOCH), were identified. We compared their clinical, virologic, and immunologic features vs 20 patients with HIV-associated diffuse large B-cell lymphoma and 19 patients with symptomatic interleukin (IL)-6 related KSHV-associated multicentric Castleman disease. Survival analyses of treated patients with PEL were then performed to identify prognostic factors and cancer-specific mortality. Compared with HIV-associated diffuse large B-cell lymphoma, PEL was associated with significant hypoalbuminemia (P < .0027), thrombocytopenia (P = .0045), and elevated IL-10 levels (P < .0001). There were no significant differences in these parameters between PEL and KSHV-associated multicentric Castleman disease. Median overall survival in treated patients with PEL was 22 months, with a plateau in survival noted after 2 years. Three-year cancer-specific survival was 47%. EBV-positive tumor status was associated with improved survival (hazard ratio, 0.27; P = .038), and elevated IL-6 level was associated with inferior survival (hazard ratio, 6.1; P = .024). Our analysis shows that IL-6 and IL-10 levels contribute to the natural history of PEL. Inflammatory cytokines and tumor EBV status are the strongest prognostic factors. Pathogenesis-directed first-line regimens are needed to improve overall survival in PEL. |
Databáze: | OpenAIRE |
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