Pulmonary Lymphangiomatosis

Abstract

Lymphangiomatosis is a rare disease characterized by diffuse infiltration of lymphangiomas in the lung, bone, and other tissues. Due to its rarity, the spectrum of lymphangiomatosis is beginning to be elucidated based on case reports. The limited pathological, radiological, and clinical studies have shed light on this disease. Treatments have been tested in unblinded manner with promising results; however, further understanding of the pathogenesis of disease, as well as its natural history, is needed to facilitate drug development.

A rare case of lymphangiomatosis

“…[1,2,7,12] The disease is more rapidly progressive in children, where the clinical features reported include chronic cough, recurrent respiratory tract infections, haemoptysis from vessel erosion, and chyloptysis. [1,3,12] Dyspnoea, chest pain, and chylous pleural effusions, are other associated features. The chylous effusions can be large, causing airway compromise.…”

“…[4] Pulmonary function tests were shown to reveal both restrictive and obstructive patterns. [3,7] This investigation is difficult in the young child. Treatment options are varied, and no single therapy has proven to be successful.…”

“…The clinical manifestations are diverse, and can occur as an isolated disorder, like a cystic hygroma, or can present with profound cardiovascular involvement, chylothoraces, and chylous pericardial effusions. [3] The diagnosis is made on a biopsy of the involved site, and demonstration of dilated lymphatic channels, with positive staining for D2-40 and CD-31 antigens. [4] Since the pathophysiology of the disease is poorly understood, therapy is largely experimental, with variable outcomes.…”

Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis)

“…Nonspecific symptoms sometimes occur, such as mild wheezing, nonproductive cough, chest pain, chest tightness, shortness of breath, dyspnoea and the disease is often misdiagnosed as asthma or other respiratory illnesses [24]. Sometimes massive pleural effusion and progressive pulmonary infiltration cause respiratory failure [15].…”

“…However, in contrast to cells involved in LAM, they lack oestrogen receptor and glycoprotein gp100, which reacts with the monoclonal antibody HMB-45 [3,23] (figure 1b). Usually, progesterone receptor is not found [24].…”