Pulmonary lymphangiomatosis: a report of two cases.
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 pulmonary 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.
de Lima
Adma Silva
AS
Universidade Federal do Paraná, Curitiba, PR, Brasil. funpul@hc.ufpr.br <funpul@hc.ufpr.br>
Martynychen
Mariane Gonçalves
MG
Florêncio
Roseni Teresinha
RT
Rabello
Lêda Maria
LM
de Barros
João Adriano
JA
Escuissato
Dante Luis
DL
D000328:Adult; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D001706:Biopsy; D003952:Diagnostic Imaging; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016898:Interferon-alpha / Q000627:therapeutic use; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D008208:Lymphatic System / Q000473:pathology; D010996:Pleural Effusion / Q000000981:diagnostic imaging; D011859:Radiography
D020533:Angiogenesis Inhibitors; D016898:Interferon-alpha