An adult case of lymphangiomatosis of the mediastinum, pulmonary interstitium and retroperitoneum complicated by chronic disseminated intravascular coagulation

Abstract

A An n a ad du ul lt t c ca as se e o of f I Iy ym mp ph ha an ng gi io om ma at to os si is s o of f t th he e m me ed di ia as st ti in nu um m, , p pu ul lm mo on na ar ry y i in nt te er rs st ti it ti iu um m a an nd d r re et tr ro op pe er ri it to on ne eu um m c co om mp pl li ic ca at te ed d b by y c ch hr ro on ni ic c d di is ss se em mi in na at te ed d i in nt tr ra av va as sc cu ul la ar r c co oa ag gu ul la at ti io on n K. Takahashi*, H. Takahashi*, K. Maeda*, S. Homma*, T. Uekusa**, T. Dambara*, S. Kira* ABSTRACT: Pulmonary lymphangiomatosis is a rare clinical and pathological entity which is distinct from lymphangiomyomatosis and from pulmonary lymphangiectasis. We report a case of a 20 year old man with diffuse lymphangiomatosis involving the mediastinum, lungs and retroperitoneum. The patient's intrathoracic lymphangiomatosis produced restrictive and obstructive impairment of his pulmonary function, but did not result in chylothorax or chylopericardium, which are two common manifestations of the disease. The patient's clinical course was remarkable for the concomitant development of chronic disseminated intravascular coagulation (DIC). Lymphangiomatosis involving both the mediastinum and pulmonary parenchyma is rare, and DIC is a very unusual complication of lymphangiomatosis.

Simultaneous hemopericardium, hemoperitoneum and hemothorax after complete surgical resection of mediastinal cystic lymphangioma: A case report

“…In literature, coagulopathy has been reported in association with lymphangiomatosis, and several potential mechanisms for this association have been advanced. Local production of fibrinolysin by lymphangiomas may be responsible for the coagulopathy [15]. This could explain blood effusion far from surgical site in the patient.…”

Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis)

“…On high-resolution chest computed tomography, some common characteristics are suggestive, but are not pathognomonic, of DPL [15]. One such instance is the presence of bilateral smooth thickening of the interlobular septa and bronchovascular bundles, which indicates growth of anastomosing lymphatic vessels in the interlobular and central peribronchovascular interstitium [7,[40][41][42]. Frequent patchy ground-glass opacities may be found, which, according to some authors [3,41], represent a recent haemorrhage and haemosiderin-laden macrophages in the air spaces [43].…”

“…In advanced cases, thin-walled vesicles containing chylous fluid have been found [17]. Although one case of DPL was diagnosed with transbronchial biopsy [7,43], in most cases reported in the literature, the diagnosis was made with open-lung biopsy [3,41,47]. In cases of bone involvement, a bone biopsy may be considered.…”

“…diffuse pulmonary lymphangiomatosis in the lung) or, more frequently, multiple organs [1]. Within the thorax, GLA may involve the lungs, mediastinum, heart, pleura, thoracic duct and chest wall [3][4][5][6][7]. Although Gorham-Stout disease (GSD), a rare lymphatic disorder characterised by progressive osteolysis, shares many features with GLA, it is currently considered as a different entity [2,8].…”

Mediastinal lymphangiomatosis coexisting with occult thymic carcinoma

“…Lymphangioma usually occurs in the neck and face of children, but is quite rare in the thorax of adults [3,9]. Lymphangiomatosis, a diffuse, proliferative form of lymphangioma, is typically benign, but association with serious morbidity is not uncommon [1,3,5,6,9].…”