29 KB176 KB19 KB192920262008Fikfak, NatašaGlaser, MarjanaKavalar, Rajkoštevilka:15 straniletnik:77str. I-151-I-155ISSN:1318-0347COBISSID:2917439URN:URN:NBN:SI:doc-X7XNUKH7slSlovensko zdravniško društvoZdravniški vestnikdiagnosisDiagnostikahematologijaHistiocitni nekrotizantni limfadenitisHistiocytic Necrotizing LymphadenitisKikuchi Fujimotova bolezenkrvne bolezniSigns and SymptomstherapyZdravljenjeZnaki in simptomiKikuchi Fujimotova bolezen| Kikuchi-Fujimoto disease|Background. The Kikuchi Fujimoto disease (KFD) or histiocytic necrotising lymphadenitis is a rare benign disease, more common in Japanese women. In other parts of the world and also in Europe the disease in rare. The pathogenesis is poorly understood. Patients and methods. We represent 2 patients who observed enlarged peripheral nodes, measuring up to 2 cm. The second patient had also big mediastinal tumor mass. On histological examination of a peripheral node in both patients KFD was diagnosed. In both no medical treatment was prescribed. The first patient has been followed up without any symptoms for the last two years, while in the second patient the mediastinal tumor mass became in 2 months larger. Another histological examination of the mass was performed and diffuse large anaplastic CD 20 + lymphoma was diagnosed. Conclusions. Most patients complain about enlarged cervical nodes accompanied by various symptoms, however the enlarged nodes rarely appear at other localizations. The nodes are mobile and measuring up to 2 cm. The only way to prove the disease is a histological examination of the node, which may show typical changes for the KFD: paracortical necrotic foci, surrounded by histiocytic aggregates, irregular rounded eosinophilic areas of different sizes in paracortex and cortex with the presence of numerous histiocytes, lymphocytes, immunoblasts, plasmacytoid monocytes and eosinophilic granulated cellular debris. It is typical not to find epithelioid cells and neutrophil granulocytes in the necrotic centres. The clinical picture of KFD is nonspecific and variable. The disease has to be distinguished especially from high grade lymphoma and SLE lymphadenitis. There is no specific medical treatment for KFD since the disease usually resolves spontaneouslyIzhodišča. Kikuchi Fujimotova bolezen (KFB) ali histiocitni nekrotizantni limfadenitis je redka benigna bolezen, za katero obolevajo predvsem japonske ženske. V drugih delih sveta, tudi v Evropi, je redka. Patogeneza bolezni je neznana. Bolniki in metode. Prikazana sta 2 bolnika, ki sta opazila povečane periferne bezgavke, velike do 2 cm, pri bolnici pa so našli tudi tumorsko maso v mediastinumu. Pri obeh so s histološkim pregledom periferne bezgavke ugotovili KFB. Nista prejela nobenega zdravljenja. Prvi bolnik je brez težav že več kot 2 leti, medtem ko se je pri drugi bolnici tumor v mediastinumu v 2 mesecih povečal. S ponovnom histološkim pregledom mediastinalnega tumorja so ugotovili, da ima bolnica difuzni velikocelični anaplastični CD 20+ B limfom. Zaključki. Klinična slika KFB je nespecifična in raznolika. Večina bolnikov ima povečane bezgavke na vratu, le redko najdemo povečane bezgavke drugje v telesu. Bezgavke so dobro premakljive in velike do 2 cm. Za potrditev bolezni je potrebno opraviti histološki pregled prizadete bezgavke, kjer najdemo za KFB značilne spremembe: parakortikalna nekrotična žarišča, ki jih obkrožajo agregati histiocitov, neenakomerna obkrožena eozinofilna polja različne velikosti v parakorteksu in korteksu s prisotnostjo številnih histiocitov, limfocitov, imunoblastov, plazmacitoidnih monocitov in eozinofilnim granuliranim celičnim drobirjem. Značilno je, da ne najdemo epiteloidnih celic niti makrofagov v centrih nekroze. Pomembno je, da bolezen ločimo od limfoma in limfadenitisa pri sistemskem lupusu, da bolnikov po nepotrebnem ne zdravimo oziroma jih ne spregledamo. Večina bolnikov s KFB ne potrebuje zdravljenja, saj bolezen spontano izzveniTEXTznanstveno časopisjejournalsSlovenian National E-content AggregatorNational and University Library of SloveniaSlovensko zdravniško društvo