Limfom ekstranodusnog limfnog tkiva u sastavu sluznica u timusu bolesnika sa Sjegrenovim sindromom i vaskulitisom kože
Sažetak
Uvod. Veza između Sjegrenovog sindroma (SS) i nastanka limfoma je dobro poznata. Prevalencija limfoma kod bolesnika sa SS je 5%. Limfom ekstranodusnog limfnog tkiva u sastavu sluznica (mucosa-associated lymphoid tissue -MALT) je najčešči tip limfoma kod bolesnika sa SS. MALT limfom se često razvije u želucu dok je u timusu veoma redak. Prikaz bolesnika. Prikazujemo bolesnika starog 61 godinu, bele rase, sa primarnim SS i vaskulitisom kože (VK) i MALT limfomom u timusu. Bolesnik je imao difuznu palpabilnu purpuru na koži nogu prethodne dve godine, intermitentno uvećanje leve parotidne žlezde i osećaj suvih usta. Rezultati Širmerovog testa bili su pozitivni, histopatološki nalaz biopsije labijalnih pljuvačnih žlezda pokazao je fokus skor ≥ 1, serološkim analizama pokazana je pozitivnost na autoantitela anti-Ro/SS-A i anti-La/SS-B, a histopatološki nalaz biopsije kože potvdio je leukocitoklastični vaskulitis. Dijagnoza SS sa vanžlezdanom kutanom manifestacijom bila je potvrđena. Detektovane su krioglobulinemija (Kg) i monoklonska gamopatija (MG), što je povećalo sumnju na postojanje hematološke maligne bolesti i sprovedene su dodatne dijagnostičke procedure. Kompjuterizovanom tomografijom grudnog koša utvrđena je uvećana, multicistično izmenjena masa u prednjem medijastinumu. Urađena je timektomija primenom video-asistirane torakalne hiruške intervencije. Histopatološkim nalazom tkiva potvrđeno je prisustvo tumorskog tkiva koje je po karakteristikama odgovaralo MALT limfomu u timusu. Primenjena je indukciona terapija pulsnim dozama glukokortikoida tokom tri dana koja je nastavljena srednjim dozama leka. Doze su postepeno smanjivane i uveden je hidroksihlorokin. Ova terapija se pokazala kao delotvorna protiv manifestacija SS. Postoperativno je sprovedena lokalna radioterapija. Zaključak. Kod bolesnika sa SS i VK, i sa prisustvom Kg i MG, neophodno je obratiti pažnju na eventualni razvoj MALT limfoma, uključujući i veoma retku lokalizaciju u timusu.
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