Author/Editor     Anderluh, Franc
Title     Lymphangioleiomyomatosis
Translated title     Limfangioleiomiomatoza
Type     članek
Source     Radiol Oncol
Vol. and No.     Letnik 36, št. 1
Publication year     2002
Volume     str. 41-6
Language     eng
Abstract     Background. Lymphangioleiomyomatosis is a rare disease of unknown origin, which affects women in their reproductive period. It is characterised by non-neoplastic proliferation of atypical smooth muscle cells in the lung parenchyma, lymphatic vessels and mediastinal and abdominal lymph nodes. The most common presenting symptoms are spontaneous pneumothorax, dyspnea, hemoptysis and chylothorax. Conclusions. High-resolution computed tomography (HRCT) and open lung biopsy followed by the immunohistologic studies are two diagnostic procedures with which diagnosis can be confirmed. Various treatment modalities are applied, particularly hormonal therapy, though their efficacy remain unknown. The prognosis of patients is bad.
Summary     lzhodišča. Limfangioleiomiomatoza je redka bolezen neznanega vzroka, za katero zbolevajo ženske v rodnem obdobju. Za bolezen je značilna neneoplastična proliferacija atipičnih gladkomišičnih celic v pljučnem parenhimu, limfnih žilah ter mediastinalnih in abdominalnih bezgavkah. V klinični sliki se pojavljajo spontani pnevmotoraksi, dispneja, hemoptize in hilotoraks. Zaključlti. Računalniška tomografija visoke ločljivosti in imunohistološke metode v bioptičnih vzorcih pljučnega tkiva nam omogočajo pravilno postavitev diagnoze. Načini zdravljenja so različni, običajno hormonski, vendar je zaenkrat njihov uspeh še vprašljiv, prognoza bolnikov pa slaba.
Descriptors     LYMPHANGIOMYOMATOSIS
TOMOGRAPHY, X-RAY COMPUTED
BIOPSY