TY - JOUR
T1 - Characterization of B cell lymphoma in patients with Sjögren's syndrome and hepatitis C virus infection
AU - Ramos-Casals, Manuel
AU - La Civita, Luca L.A.
AU - De Vita, Salvatore
AU - Solans, Roser
AU - Luppi, Mario
AU - Medina, Francisco
AU - Caramaschi, Paola
AU - Fadda, Patrizia
AU - De Marchi, Ginevra
AU - Lopez-Guillermo, Armando
AU - Font, Josep
AU - Brito-Zerón, Pilar
AU - Loustaud-Ratti, Veronique
AU - Zeher, Margit
AU - Szodoray, Peter
AU - Bosch, Jose Angel
AU - Selva-O'Callaghan, Albert
AU - Vilardell, Miquel
AU - Toussirot, Eric
AU - Wendling, Daniel
AU - Rosas, Jose
AU - Anaya, Juan Manuel
AU - Forns, Xavier
AU - Sanchez-Tapias, Jose
AU - Jara, Luis Javier
N1 - Copyright:
Copyright 2012 Elsevier B.V., All rights reserved.
PY - 2007/2/15
Y1 - 2007/2/15
N2 - 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).
AB - 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).
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U2 - 10.1002/art.22476
DO - 10.1002/art.22476
M3 - Article
C2 - 17266090
AN - SCOPUS:33847004729
SN - 2151-4658
VL - 57
SP - 161
EP - 170
JO - Arthritis Care and Research
JF - Arthritis Care and Research
IS - 1
ER -
