Linfangiomatose pulmonar: relato de dois casos / Pulmonary lymphangiomatosis: a report of two cases
J. bras. pneumol
; 33(2): 229-233, mar.-abr. 2007. ilus
Article
in Portuguese
| LILACS
| ID: lil-459296
Responsible library:
BR1.1
RESUMO
A linfangiomatose, doença rara e de origem controversa, ocorre em indivíduos de todas as faixas etárias, predominando entre os mais jovens, sem predileção por sexo. Comumente cursa com envolvimento torácico, porém órgãos como ossos, baço e fígado podem ser acometidos. Histologicamente, o envolvimento pulmonar cursa com proliferação, anastomoses complexas e dilatação secundária do sistema linfático. Clinicamente, a apresentação é variável. Os achados radiográficos podem ser sugestivos e o diagnóstico é definido pela histologia. Relatamos dois casos da doença; uma das pacientes era oligossintomática e encontra-se em tratamento; na outra, a doença foi mais agressiva e o diagnóstico muito tardio, culminando em óbito.
ABSTRACT
Lymphangiomatosis, a rare diseases of controversial origin, occurs in individuals of any age, regardless of gender, but is predominantly seen in younger individuals. It often presents with thoracic involvement, although, the bones, spleen and liver can also be affected. Histologically, the pulmonar involvement includes proliferation, complex anastomoses and secondary dilatation of the lymphatic vessels. Clinically, the presentation is variable. Although radiographic findings can be suggestive of the disease, the final diagnosis is made histologically. We report two cases of lymphangiomatosis, both in females one was oligosymptomatic and is being treated for the disease; the other had a more progressive form, was diagnosed quite late and ultimately died of the disease.
Full text:
Available
Collection:
International databases
Database:
LILACS
Main subject:
Lymphangioleiomyomatosis
Type of study:
Diagnostic study
Limits:
Adult
/
Female
/
Humans
Language:
Portuguese
Journal:
J. bras. pneumol
Journal subject:
Pulmonary Disease (Specialty)
Year:
2007
Document type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Hospital Nossa Senhora das Graças/BR
/
Universidade Federal do Paraná/BR