Lymphangioleiomyomatosis

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 treatmentmodalities are applied, particularly hormonal therapy, though their efficacy remain unknown. The prognosis of patients is bad.

Keywords

Biopsija;Biopsy;Diagnosis;Limfangiomiomatoza;Lymphangiomyomatosis;Pathology;Therapy;Tomografija radiografska, računalniška;Tomography, X-ray computed

Data

Language:	English
Year of publishing:	2002
Typology:	1.02 - Review Article
Organization:	OI - Institute of Oncology
Publisher:	Slovenian Medical Association - Slovenian Association of Radiology, Nuclear Medicine Society, Slovenian Society far Radiotherapy and Oncology, and Slovenian Cancer Society
UDC:	616-006
COBISS:	14747865
ISSN:	1318-2099
Views:	10
Downloads:	2
Average score:	0 (0 votes)
Metadata:

Other data

Secondary language:	Slovenian
Secondary title:	Limfangioleiomiomatoza
Secondary abstract:	lzhodišča. Limfangioleiomiomatoza je redka bolezen neznanega vzroka, za katerozbolevajo ženske v rodnem obdobju. Za bolezen je značilna neneoplastičnaproliferacija 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.
Secondary keywords:	Lymphangiomyomatosis;Diagnosis;Therapy;Pathology;Tomography, X-ray computed;Biopsy;Limfangiomiomatoza;Biopsija;Tomografija radiografska, računalniška;
Source comment:	BSDOCID88061;
Pages:	str. 41-46
Volume:	ǂLetn. ǂ36
Issue:	ǂšt. ǂ1
Chronology:	2002
ID:	22754332