Malt lymphoma associated with Sjögren Syndrome: clinical report
Keywords:
Sjögren Syndrome, B cells Lymphoma, MALT Lymphoma, Parotid gland, XerostomiaAbstract
Sjögren 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 with
systemic manifestations, which include the B cells lymphoma,
namely the MALT lymphoma of the Sjögren’ 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 to the 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,
and their positivities confirm the Primary Sjögren 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.
Downloads
References
Vitali C, Bombardieri S, Moutsoupoulos HM. The European Community Study Group On Diagnostic Criteria For Sjögren’s Syndrome-Preliminary criteria for Sjögren’s Syndrome. Arthritis Rheum 1993; 36:340-347.
Bell M, Askari A, Bookman A, Frydrych S, Lamont J, McComb J, Muscoplat C, Slomovic A. Sjögren’s Syndrome: a critical review of clinical management. J Rheumatol 1999; 26:2051-2061.
Tapinos NI, Polihronis M, Tzioufas AG, Motsopoulos HM. Sjögren’s Syndrome: autoimmune epithelitis. Adv Exp Med Biol 1999: 455 : 127-134.
Manganelli P, Fietta P. Apoptosis and Sjögren’s Syndrome. Semin Arthritis Rheum 2003 ;33(1):49-65.
Carsons S. A review and update of Sjögren’s Syndrome: manifestations, diagnosis and treatment. Am J Manag Care 2001;7: 433-443.
Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N,
Weisman MH; European Study Group on Classification Criteria for Sjogren’s Syndrome. Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61(6):554-558.
Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LT. Lymphoma and other malignancies in primary Sjögren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis 2006; 65(6):796-803.
Zulman J, Jaffe R, Talal N. Evidence that the malignant lymphoma of Sjögren’s Syndrome is a monoclonal B cell neoplasm. N Engl J Med 1978; 299; 1215-1220.
Skopouli FN, Dafni U, Ionnidis JP, Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjögren’s Syndrome. Semin Arthritis Rheum 2000;29: 296-304.
Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjögren’s syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren’s Syndrome. Arthritis Rheum 1999;42:1765 -1772.
Royer B, Cazals-Hatem D, Sibilia J et al. Lymphomas in patients with Sjögren’s syndrome are marginal zone B-cell neoplasms, arise in diverse extranodal and nodal sites, and are not associated with viruses. Blood 1997;90:766 -775.
Stewart A, Blenkinsopp PT, Henry K. Bilateral parotid mucosa-associated lymphoid tissue lymphoma and Sjögren’s syndrome. Br J Oral Maxillofac Surg 1994;32:318 -322.
Tonami H et al. Mucosa-associated lymphoid tissue lymphoma in Sjogren’s syndrome: initial and follow-up imaging features. Am J Roentgenol 2002;179(2):485-489.
Ambrosetti A et al. Most cases of primary salivary mucosa-associated lymphoid tissue lymphoma are associated either with Sjögren syndrome or hepatitis C virus infection. Br J Haematol 2004 ;126(1):43-49.
Additional Files
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2023 Medicina Interna
Open Access
