Treatment of mucosa-associated lymphoid tissue lymphoma in Sjogren's syndrome: A retrospective clinical study
Journal of Rheumatology, 08/19/2011
Clinical Article
Pollard RPE et al. – An initially high SS disease activity likely constitutes an adverse prognostic factor for progression of lymphoma and/or SS. Such patients may require treatment for both MALT lymphoma and SS. In SS patients with localized asymptomatic MALT lymphoma and low SS disease activity, a “watchful waiting” strategy seems justified.
Methods- All consecutive patients with SS and MALT lymphoma (MALT-SS) diagnosed in the University Medical Center Groningen between January 1997 and January 2009 were analyzed
- Clinical course and treatment outcome of SS and MALT lymphoma evaluated
- From a total of 329 patients with SS, 35 MALT-SS patients were identified, with a median followup of 76 months (range 16–153 mo)
- MALT lymphoma localized in parotid gland in all cases
- Treatment consisted of “watchful waiting” (n = 10), surgery (n = 3), radiotherapy (n = 1), surgery combined with radiotherapy (n = 2), rituximab only (n = 13), or rituximab combined with chemotherapy (n = 6)
- Complete response observed in 14 patients, partial response in 1 patient, and stable disease in 20 patients
- In 6 of 7 patients with initially high SS disease activity (M-protein, cryoglobulins, IgM rheumatoid factor > 100 KIU/l, severe extraglandular manifestations), MALT lymphoma progressed and/or SS disease activity increased after a median followup of 39 months (range 4–98 mo), necessitating retreatment
- Only 1 patient with MALT who had low SS disease activity showed progression of lymphoma when left untreated







