Marginal zone B-cell lymphoma with multiple extranodal locations in a patient with Sjögren’s syndrome – a diagnostic problem
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Submission date: 2014-04-09
Final revision date: 2014-07-28
Acceptance date: 2014-07-30
Online publication date: 2014-09-12
Publication date: 2014-08-31
Reumatologia 2014;52(4):276-281
Sjögren’s syndrome is a chronic autoimmune disease characterized by the presence of lymphocytic infiltrates in exocrine glands, mainly salivary and lacrimal glands, which result in xerophthalmia and xerostomia. About half of the patients develop systemic complications, including lymphoproliferative disorders.
We report a case of a 27-year-old woman with a diagnosis of Sjögren’s syndrome and a suspicion of respiratory system involvement in the course of granulomatosis with polyangiitis. Histopathological examination of a skin lesion suggested marginal zone B-cell lymphoma. After pathological and immunohistochemical evaluation of all available previous biopsy samples and the medical documentation the diagnosis of extranodal marginal zone B-cell lymphoma stage IV according to the Ann Arbor classification was rendered. The patient was referred to the Department of Haematology and was treated with R-CVP (cyclophosphamide, vincristine, prednisone, rituximab).
Bowman SJ, Ibrahim GH, Holmes G, et al. Estimating the prevalence among Caucasian women of primary Sjögren’s syndrome in two general practices in Birmingham UK. Scand J Rheumatol 2004; 33: 39-43. .
Pillemer SR, Matteson EL, Jacobsson LT, et al. Incidence of physician-diagnosed primary Sjögren syndrome in residents of Olmsted County, Minnesota. Mayo Clin Proc 2001; 76: 593-599. .
Westhoff G, Zink A. Epidemiology of primary Sjörgren’s syndrome. J Rheumatol 2010; 69: 41-49. .
Dafni UG, Tzioufas AG, Staikos P, et al. Prevalence of Sjögren’s syndrome in a closed rural community. Ann Rheum Dis 1997; 56: 521-525. .
Ioannidis JP, Vassiliou VA, Moutsopoulos HM. Long-term risk of mortality and limfoproliferative disease and predictive classification of primary Sjögren’s syndrome. Arthritis Rheum 2002; 46: 741-747. .
Lazarus MN, Robinson D, Mak V, et al. Incidence of cancer in a cohort of patients with primary Sjögren’s syndrome. Rheumatology 2006; 45: 1012-1015. .
Kassan SS, Thomas TL, Moutsopoulos HM, et al. Increased risk of lymphoma in sicca syndrome. Ann Intern Med 1978; 89: 888-892. .
Gasparotto D, De Vita S, De Re V, et al. Extrasalivary lymphoma development in Sjögren’s syndrome. Arthritis Rheum 2003; 48: 3181-3186. .
Engels EA, Cerhan JR, Linet MS, et al. Immune-related conditions and immune-modulating medications as risk factors for non-Hodgkin’s lymphoma: a case-control study. Am J Epidemiol 2005; 162: 1153-1161. .
Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjögren’s syndrome. Arthritis Rheum 1999; 42: 1765-1772.
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