Dr. Font is deceased.
Characterization of B cell lymphoma in patients with Sjögren's syndrome and hepatitis C virus infection
Article first published online: 31 JAN 2007
Copyright © 2007 by the American College of Rheumatology
Arthritis Care & Research
Volume 57, Issue 1, pages 161–170, 15 February 2007
How to Cite
Ramos-Casals, M., Civita, L. l., de Vita, S., Solans, R., Luppi, M., Medina, F., Caramaschi, P., Fadda, P., de Marchi, G., Lopez-Guillermo, A. and Font, J. (2007), Characterization of B cell lymphoma in patients with Sjögren's syndrome and hepatitis C virus infection. Arthritis & Rheumatism, 57: 161–170. doi: 10.1002/art.22476
- Issue published online: 31 JAN 2007
- Article first published online: 31 JAN 2007
- Manuscript Accepted: 9 MAR 2006
- Manuscript Received: 9 JAN 2006
- Fondo de Investigación Sanitaria. Grant Number: 04/0701
- Sjögren's syndrome;
- Hepatitis C virus;
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.
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.
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.
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).