Sjögren-Syndrom - können wir das Lymphomrisiko stratifizieren?

Zusammenfassung

Patienten mit Sjögren-Syndrom, insbesondere der primären Form, weisen gegenüber der Normalbevölkerung ein deutlich erhöhtes Lymphomrisiko auf. Bei diesen Lymphomen handelt es sich in der Regel zunächst um niedrig-maligne (indolente) B-Zell-Lymphome, die bevorzugt in den durch das Sjögren-Syndrom betroffenen extranodalen Geweben oder korrespondierenden Lymphknoten entstehen. Obwohl nur sehr begrenzte Untersuchungen bezüglich einer Risikostratifizierung der Lymphomentwicklung vorliegen, betreffen diese offenbar häufiger Patienten mit begleitenden extraglandulären Manifestationen des Sjögren-Syndroms, einer Gammopathie bzw. Kryoglobulinämie und/oder erniedrigtem Serum-C4-Komplementspiegel. Diese Patienten sollten daher im Verlauf besonders sorgfältig auf potenzielle Hinweise auf eine Lymphommanifestation, wie die persistierende Schwellung exokriner Drüsen oder eine Lymphadenopathie, untersucht werden. Therapeutisch finden die Protokolle der zugrunde liegenden Lymphomentität Anwendung, wobei durch Kombinationen von Chemo- und (Radio-)Immuntherapie auch hier vielversprechende Behandlungsfortschritte vorliegen.

Abstract

Patients with primary Sjögren’s syndrome - in contrast to patients with secondary Sjögren’s syndrome - are at a significantly increased risk of developing a low-grade non-Hodgkin’s lymphoma compared to the general population. These lymphomas are almost exclusively of B-cell origin and usually arise in tissues affected by the underlying chronic inflammatory process. Though the investigations concerning risk stratification are quite limited, it seems that these lymphomas preferentially affect patients with extraglandular manifestations of Sjögren’s syndrome as well as patients with associated gammopathy, cryoglobulinemia and/or reduced C4 level. Consequently, these patients should be carefully checked for evidence of lymphoma, such as persistent glandular swelling or lymphadenopathy. The therapeutic options of overt Sjögren’s-associated lymphoma follow the respective lymphoma protocols. Recent advances have been reported by combining chemo- and immunotherapy, although only limited data are available from affected Sjögren’s patients.

Schlüsselwörter

Sjögren-Syndrom - niedrig-maligne B-Zell-Lymphome - Chemotherapie - Immuntherapie

Key words

Sjögren’s syndrome - low-grade Non-Hodgkin’s lymphoma - chemotherapy - immunotherapy

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