Marginal zone B-cell lymphoma with multiple extranodal locations in a patient with Sjögren’s syndrome – a diagnostic problem

Autor: Marta Domżalska, Żaneta Smoleńska, Zenobia Czuszyńska, Katarzyna Gałecka, Wojciech Biernat, Andrzej Mital, Zbigniew Zdrojewski
Jazyk: angličtina
Rok vydání: 2014
Předmět:
Zdroj: Rheumatology, Vol 52, Iss 4, Pp 276-281 (2014)
Druh dokumentu: article
ISSN: 0034-6233
2084-9834
DOI: 10.5114/reum.2014.44708
Popis: 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).
Databáze: Directory of Open Access Journals