Linfangiomatose pulmonar: relato de dois casos

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 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.

Diffuse pulmonary lymphangiomatosis

“…On chest CT scans, the findings include thickening of the interlobular septa and bronchovascular bundles, patchy ground-glass opacities, diffuse infiltration of mediastinal and hilar soft tissue, and pleural effusion. (1,2,4) In our case, the imaging findings were thickening of the interlobular septa and bronchovascular bundles, mediastinal and pleural infiltration, and associated pleural effusion, all of which are compatible with DPL. The major differential diagnosis is pulmonary lymphangiectasia, a rare condition characterized by diffuse dilatation of the pulmonary lymphatics.…”

“…Most symptoms are mild; patients present with cough, shortness of breath, and hemoptysis (with or without chylous effusion), pleuropericardial effusion, and pneumothorax. (1)(2)(3)(4) The proliferation of lymphatic channels explains the most common thoracic imaging findings of DPL. On chest CT scans, the findings include thickening of the interlobular septa and bronchovascular bundles, patchy ground-glass opacities, diffuse infiltration of mediastinal and hilar soft tissue, and pleural effusion.…”

Characteristics of Patients with Lymphangioleiomyomatosis and Pleural Effusion: A Systematic Review

“…Figure 1 presents a flowchart showing the complete breakdown of the steps used in the identification of appropriate studies [9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97,98,99,100,101,…”

“…Cytology and culture of PE were negative in all cases. In 44 of 69 patients (63.8%) in whom ascites was described, PE was accompanied by this condition [9,11,16,17,18,24,26,28,30,39,48,52,55,58,63,64,65,68,73,75,76,78,82,84,85,86,88,95], and in 97.3% (36/37) of the cases it was chylous. The only case of nonchylous ascites corresponded to the already mentioned patient that had a hemiperitoneum and a serous-hematic PE [48].…”

Rare cause of paradoxical worsening of pleural effusion in a patient with tuberculosis

“…Nonmalignant etiology is separated into congenital, idiopathic, and miscellaneous. Miscellaneous causes include Castleman's disease, lymphangioleiomyomatosis,[3] sarcoidosis, Kaposi sarcoma, Noonan syndrome, Yellow nail syndrome, TB,[4] Down syndrome, congenital lymphangiectasia, Waldenstrom macroglobulinemia, filariasis, thoracic irradiation, subclavian vein thrombosis, constrictive pericarditis, and cirrhosis. [5]…”