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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Dr. med. Arne Hansen
Medizinische Poliklinik CCM, Charité Universitätsmedizin Berlin
Luisenstraße 11 - 13A
10117 Berlin
Telefon: 0 30/4 50-51 40 12
Fax: 0 30/4 50-51 49 03
eMail: arne.hansen@charite.de
