title: Incidence of lymphoma in systemic sclerosis: a retrospective analysis of 218 Hungarian patients with systemic sclerosis.
abstract: Recent results suggest that B cells may have multiple pathogenic roles in systemic sclerosis (SSc) and there may be increased incidence of B cell lymphomas in SSc. Here, we assessed the prevalence of lymphomas in a large SSc cohort. We analyzed data of 218 Hungarian patients undergoing follow-ups in our institutions between 1995 and 2007. During this follow-up period, there were three SSc patients, who eventually developed B cell lymphoma. The first case is a woman with diffuse cutaneous form of SSc (dcSSc) including pulmonary, cardiac, gastrointestinal, and renal manifestations and anti-topoisomerase I antibody positivity. B cell chronic lymphocytic leukemia (B-CLL) with Zap70 expression (Rai I stage) developed 2 years after the onset of SSc. The second case is a woman with dcSSc presenting with pulmonary, cardiac, and gastroesophageal manifestations. Twenty-one months after disease onset, a chronic small lymphocytic B cell non-Hodgkin's lymphoma was diagnosed from retroperitoneal lymph nodes. Our third case is a woman with dcSSc and no internal organ manifestations. She also developed Zap70-positive B-CLL, stage Rai I 9 months after the onset of SSc. Thus, there were three cases of B cell lymphoma among our 218 SSc patients (1.38%). The association of scleroderma and non-Hodgkin's lymphoma may be a rather uncommon feature; however, the incidence of lymphoma among Hungarian SSc patients may be 1.9-2.5 times higher than that in the general population. In our three patients, B cell lymphoma developed within 2 years after the onset of SSc. Altered B cell function implicated in the pathogenesis of SSc may lead to the development of lymphoid malignancies.
authors.affiliation: Department of Oncology, University of Debrecen Medical Center, Debrecen, Hungary.
authors.forename: Eva
authors.initials: E
authors.lastname: Szekanecz
authors.forename: Szilvia
authors.initials: S
authors.lastname: Szamosi
authors.forename: Lajos
authors.initials: L
authors.lastname: Gergely
authors.forename: Péter
authors.initials: P
authors.lastname: Keszthelyi
authors.forename: Zoltán
authors.initials: Z
authors.lastname: Szekanecz
authors.forename: Gabriella
authors.initials: G
authors.lastname: Szucs
mesh_terms: D000368:Aged; D005260:Female; D006801:Humans; D006814:Hungary / Q000453:epidemiology; D016393:Lymphoma, B-Cell / Q000150:complications* / Q000453:epidemiology; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012595:Scleroderma, Systemic