title: Treatment of mucosa-associated lymphoid tissue lymphoma in Sjogren's syndrome: a retrospective clinical study.
abstract: OBJECTIVE
  To retrospectively analyze the clinical course of patients with mucosa-associated lymphoid tissue (MALT)-type lymphoma of the parotid gland and associated Sjögren's syndrome (SS).
  
  
  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 were evaluated.
  
  
  RESULTS
  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 was localized in the 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 was 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Hematology, University Medical Center Groningen, University of Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands.
authors.forename: Rodney P E
authors.initials: RP
authors.lastname: Pollard
authors.forename: Justin
authors.initials: J
authors.lastname: Pijpe
authors.forename: Hendrika
authors.initials: H
authors.lastname: Bootsma
authors.forename: Fred K L
authors.initials: FK
authors.lastname: Spijkervet
authors.forename: Philip M
authors.initials: PM
authors.lastname: Kluin
authors.forename: Jan L N
authors.initials: JL
authors.lastname: Roodenburg
authors.forename: Cees G M
authors.initials: CG
authors.lastname: Kallenberg
authors.forename: Arjan
authors.initials: A
authors.lastname: Vissink
authors.forename: Gustaaf W
authors.initials: GW
authors.lastname: van Imhoff
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D010306:Parotid Gland / Q000473:pathology / Q000601:surgery; D010307:Parotid Neoplasms / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D012189:Retrospective Studies; D000069283:Rituximab; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology; D016896:Treatment Outcome; D057832:Watchful Waiting
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D000069283:Rituximab