Reumatizam, Vol. 68 No. 2, 2021.
Review article
https://doi.org/10.33004/reumatizam-68-2-3
Indolent extranodal B-NHL CD20 positive MALT lymphoma in a patient with primary Sjögren’s syndrome with a good response to rituximab treatment
Nadica Laktašić Žerjavić
Mislav Pap
Iva Žagar
Kristina Kovač Durmiš
Porin Perić
Abstract
Sjögren’s syndrome (SS) is a chronic autoimmune disease in which chronic lymphocytic infiltration leads to progressive destruction of the exocrine glands and consequently causes the dry syndrome. The most commonly affected are the lacrimal and salivary glands. Less often, SS presents with the symptoms of exocrine glands of other localisation, arthritis, myositis, vasculitis involving the skin and the nervous system, interstitial lung disease, and kidney disease. The most serious complication is the development of lymphoproliferative disease, primarily non-Hodgkin’s lymphoma (NHL). We present the case of a patient who developed indolent extranodal B-NHL CD20-positive MALT -type lymphoma after 23 years of primary SS duration, with a good response to rituximab treatment.
Keywords
Sjögren’s Syndrome, Lymphoproliferative Disorders, B-Cell Marginal Zone Lymphoma, Rituximab
Hrčak ID:
287143
URI
Publication date:
4.12.2022.
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