Indolent extranodal B-NHL CD20 positive MALT lymphoma in a patient with primary Sjögren’s syndrome with a good response to rituximab treatment
Authors:
Nadica Laktašić Žerjavić, Mislav Pap, Iva Žagar, Kristina Kovač Durmiš, Porin Perić
Summary
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.
Sažetak
Sjögrenov sindrom (SS) kronična je autoimuna bolest u kojoj limfocitna infiltracija dovodi do progresivnog oštećenja egzokrinih žlijezda i sljedstvenoga suhog sindroma. Najčešće zahvaća suzne i slinovne žlijezde, a manje često se javljaju simptomi egzokrinih žlijezda druge lokalizacije, artritis, miozitis, vaskulitis sa zahvaćanjem kože i živčanog sustava, intersticijska bolest pluća i bubrežna bolest. Najteža komplikacija je razvoj limfoproliferativne bolesti, u prvom redu ne-Hodgkinovog limfoma (NHL-a). U radu prikazujemo bolesnicu koja je nakon 23 godine trajanja primarnog SS-a razvila indolentni ekstranodalni B-NHL CD20 pozitivni limfom tipa MALT uz dobar odgovor na liječenje rituksimabom.
Vol.: Reumatizam 2021;68(2):86–95
