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Triple Association Between Hepatitis C Virus Infection, Systemic Autoimmune Diseases, and B Cell Lymphoma
MANUEL RAMOS-CASALS, OLGA TREJO, MARIO GARCÍA-CARRASCO, RICARD CERVERA, GLORIA DE LA RED, VICTOR GIL, ARMANDO LÓPEZ-GUILLERMO, MIGUEL INGELMO, and JOSEP FONT
ABSTRACT. Objective. To analyze the clinical characteristics of patients from a Department of Autoimmune Diseases presenting chronic hepatitis C virus (HCV) infection, systemic autoimmune disease, and B cell lymphoma. Methods. We analyzed the records of 100 consecutive patients with systemic autoimmune diseases and associated HCV infection seen in our department between 1994 and 2000. We retrospectively investigated the development of B cell malignancies after the diagnosis of HCV related autoimmune disease. Results. Six patients with HCV related systemic autoimmune disease presented B cell non-Hodgkin's lymphoma (NHL). These patients fulfilled the diagnostic criteria for Sjögren's syndrome (n = 4) and polyarteritis nodosa (PAN; n = 2). Four patients were female and 2 male, with a mean age at lymphoma diagnosis of 62 years (range 45–78). The main immunologic markers were hypocomplementemia in all patients and cryoglobulinemia in 5 (83%). Primary extranodal localization of lymphoma was observed in 3 (50%) patients: prostate (n = 1), liver and ovary (n = 1), and ocular annexa (n = 1). Clinically, NHL was classified as indolent lymphoma in 3 patients and aggressive lymphoma in 3. NHL histologic types were diffuse large cell lymphoma (n = 4), extranodal marginal zone B cell lymphoma (n = 1), MALT lymphoma (n = 1), and lymphoplasmacytic lymphoma (n = 1). Conclusion. We describe 6 patients with a triple association of HCV infection, systemic autoimmune disease, and NHL. Characteristics of these patients included a high prevalence of cryoglobulinemia (that clearly contributes to fulfilment of diagnostic criteria for PAN) and an elevated frequency of primary extranodal involvement. We recommend careful evaluation of patients with B cell NHL to detect silent autoimmune or chronic viral diseases. This triple association reinforces the suspected links between autoimmunity, infection, and cancer. (J Rheumatol 2004;31:495-9) Key Indexing Terms:
CRYOGLOBULINEMIA
From the Departments of Autoimmune Diseases and Hematology, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, University of Barcelona, Barcelona, Spain; and Rheumatology Unit, Benemérita Universidad Autonoma de Puebla, Puebla, México. Dr. Trejo is a Research Fellow from the Hospital Clínic (Beca Premi Fi de Residènicia 2001). M. Ramos-Casals, MD, PhD; O. Trejo, MD, PhD; R. Cervera, MD, PhD; G. De La Red, MD; V. Gil, MD; M. Ingelmo, MD, PhD; J. Font, MD, PhD, Department of Autoimmune Diseases; A. López-Guillermo, MD, PhD, Department of Hematology, IDIBAPS; M. García-Carrasco, MD, PhD, Department of Autoimmune Diseases, IDIBAPS, Rheumatology Unit, Benemérita Universidad Autonoma de Puebla. Address reprint requests to Dr. M. Ramos-Casals, Servei de Malalties Autoimmunes, Hospital Clínic, C/Villarroel 170, 08036 Barcelona, Spain. E-mail: mramos@clinic.ub.es Submitted December 23, 2002; revision accepted August 12, 2003. |