Malt lymphoma associated with Sjögren Syndrome: clinical report

Sjogren Syndrome is considered an autoimmune disease of the exocrine glands, which involves especially the salivary and lachrymal glands. The clinical manifestations are wide, and can only be clearly shown by dryness of the oral and conjunctival mucosa or by articular, lung and/or renal involvement. One third of the patients with this syndrome present systemic manifestations including B cells lymphoma, namely the MALT lymphoma of the Sjogren’ target-organs. In spite of its scarcity and its slow course, the patients have a worst prognosis in relation to the other type of MALT lymphoma, due to its tendency to dissemination and inherent therapeutic implications. A case of a 64 years old male patient coming from the Oncology consultation is reported, with MALT lymphoma initial diagnosis, shown after the right parotid gland excision which was due to recurrent parotiditis episodes. Once the patient presented oral and conjunctival dryness complaints, SS-A(Ro) e SS-B(La) autoantigens were determined and Schirmer’s I test was performed, its positive values confirm the Primary Sjogren Syndrome diagnosis. Due to xerostomia and MALT lymphoma coexistence, chemotherapy treatment with CHOP protocol was chosen. The scarcity and singularity of this case study have led the authors to its publication.