Incidence of lymphoma in systemic sclerosis: a retrospective analysis of 218 Hungarian patients with systemic sclerosis.

Szekanecz, E [Szekanecz, Éva (klinikai onkológi...), szerző] Onkológiai Intézet (DE / ÁOK); Szamosi, S [Szamosi, Szilvia (belgyógyászat, re...), szerző]; Gergely, L [Gergely, Lajos (Hematológia, Immu...), szerző] C épület (DE / KK / BelgyKL); Belgyógyászati Intézet (DE / ÁOK); Keszthelyi, P; Szekanecz, Z [Szekanecz, Zoltán (Reumatológia), szerző] C épület (DE / KK / BelgyKL); Szucs, G [Szűcs, Gabriella (Reumatológia, all...), szerző] Általános Orvostudományi Kar (DE)

Angol nyelvű Szakcikk (Folyóiratcikk) Tudományos
Megjelent: CLINICAL RHEUMATOLOGY 0770-3198 1434-9949 27 (9) pp. 1163-1166 2008
  • SJR Scopus - Medicine (miscellaneous): Q2
Azonosítók
Szakterületek:
  • Egyéb orvostudományok
  • Klinikai orvostan
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.
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2025-02-12 14:14