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 |