Characterization of B cell lymphoma in patients with Sjögren's syndrome and hepatitis C virus infection.
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.


CONCLUSIONS
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).
Ramos-Casals
Manuel
M
Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, School of Medicine, University of Barcelona, Barcelona, Spain. mramos@clinic.ub.es
la Civita
Luca
L
de Vita
Salvatore
S
Solans
Roser
R
Luppi
Mario
M
Medina
Francisco
F
Caramaschi
Paola
P
Fadda
Patrizia
P
de Marchi
Ginevra
G
Lopez-Guillermo
Armando
A
Font
Josep
J
D000328:Adult; D000368:Aged; D015551:Autoimmunity / Q000276:immunology; D003450:Cryoglobulins / Q000378:metabolism; D005088:Exocrine Glands / Q000473:pathology / Q000821:virology; D005260:Female; D016174:Hepacivirus / Q000472:pathogenicity; D006526:Hepatitis C / Q000150:complications* / Q000276:immunology* / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology / Q000821:virology; D016393:Lymphoma, B-Cell / Q000276:immunology* / Q000378:metabolism / Q000821:virology*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis / Q000209:etiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012217:Rheumatoid Factor / Q000097:blood; D012859:Sjogren's Syndrome / Q000150:complications / Q000276:immunology* / Q000378:metabolism / Q000821:virology*; D013270:Stomach / Q000473:pathology / Q000821:virology; D016019:Survival Analysis; D016896:Treatment Outcome
D003450:Cryoglobulins; D012217:Rheumatoid Factor