1. Characterization of B cell lymphoma in patients with Sjögren's syndrome and hepatitis C virus infection.
- Author
-
Ramos-Casals M, la Civita L, de Vita S, Solans R, Luppi M, Medina F, Caramaschi P, Fadda P, de Marchi G, Lopez-Guillermo A, and Font J
- Subjects
- Adult, Aged, Autoimmunity immunology, Cryoglobulins metabolism, Exocrine Glands pathology, Exocrine Glands virology, Female, Hepacivirus pathogenicity, Hepatitis C pathology, Humans, Liver pathology, Liver virology, Lymphoma, B-Cell metabolism, Lymphoma, B-Cell, Marginal Zone diagnosis, Lymphoma, B-Cell, Marginal Zone etiology, Lymphoma, B-Cell, Marginal Zone pathology, Male, Middle Aged, Prognosis, Rheumatoid Factor blood, Sjogren's Syndrome complications, Sjogren's Syndrome metabolism, Stomach pathology, Stomach virology, Survival Analysis, Treatment Outcome, Hepatitis C complications, Hepatitis C immunology, Lymphoma, B-Cell immunology, Lymphoma, B-Cell virology, Sjogren's Syndrome immunology, Sjogren's Syndrome virology
- Abstract
Objective: To characterize the clinical and immunologic patterns of expression, response to therapy, and outcome of patients with Sjögren's syndrome (SS) and associated hepatitis C virus (HCV) infection who developed B cell lymphoma., Methods: Various international reference centers constituted a multicenter study group with the purpose of creating a registry of patients with SS-HCV who developed B cell lymphoma. A protocol form was used to record the main characteristics of SS, chronic HCV infection, and B cell lymphoma., Results: Twenty-five patients with SS-HCV with B cell lymphoma were included in the registry. There were 22 (88%) women and 3 (12%) men (mean age 55, 58, and 61 years at SS, HCV infection, and lymphoma diagnosis, respectively). The main extraglandular SS manifestations were cutaneous vasculitis in 15 (60%) patients and peripheral neuropathy in 12 (48%); the main immunologic features were positive rheumatoid factor (RF) in 24 (96%) and type II cryoglobulins in 20 (80%). The main histologic subtypes were mucosa-associated lymphoid tissue (MALT) lymphoma in 11 (44%) patients, diffuse large B cell lymphoma in 6 (24%), and follicular center cell lymphoma in 6 (24%). Fifteen (60%) patients had an extranodal primary location, most frequently in the parotid gland (5 patients), liver (4 patients), and stomach (4 patients). Twelve (52%) of 23 patients died after a median followup from the time of lymphoma diagnosis of 4 years, with lymphoma progression being the most frequent cause of death. Survival differed significantly between the main types of B cell lymphoma., Conclusion: Patients with SS-HCV and B cell lymphoma are clinically characterized by a high frequency of parotid enlargement and vasculitis, an immunologic pattern overwhelmingly dominated by the presence of RF and mixed type II cryoglobulins, a predominance of MALT lymphomas, and an elevated frequency of primary extranodal involvement in organs in which HCV replicates (exocrine glands, liver, and stomach).
- Published
- 2007
- Full Text
- View/download PDF