Diffuse pulmonary lymphangiomatosis: A rare case report in an adult

Abstract

Rationale: Diffuse pulmonary lymphangiomatos (DPL) is a rare aggressive lymphatic disorder characterized by proliferation of anastomozing lymphatic vessels and extremely rare in adult patients.

Patient concerns: We report a case of diffuse pulmonary lymphangiomatosis in 59-year-old man presented with cough and sputum for 2 months.

Diagnoses: Combining clinical manifestations with results of radiological, bronchoscopy, and surgical lung biopsy, it was consistent with the diagnosis of DPL.

Interventions: After bronchoalveolar lavage and biopsy, symptom of cough got worse suddenly accompanied by excessive chyloptysis. The patient received an emergency surgical intervention and low fat medium chain fat treatment.

Outcomes: The patient was discharged with a much better health condition.

Lessons: This case report is the oldest patient reported in the English literature, to the best of our knowledge. Serious complications of bronchoscopy should be considered, especially in DPL patients with severely enlarged mediastinum or with thin-walled translucent vesicles under endoscopy.

Figure 1

A–B, Chest CT demonstrates diffuse thickening of the interlobular septa, patch opacity and ground-glass lesions in both lungs (A); CT also reveals mediastinal enlargemengt without enhancement and bilateral pleural effusion (B). C–D, A bronchoscopy shows edematous changes and thin-walled translucent vesicles distributed in a diffuse way. E–F, Histology of the lung tissue showing proliferation of irregular lymphatic vessels, lined by single layer of endothelial cells lacking cytological atypia (E, HE, original magnification ×100; F, immunohistochemical staining with D2–40 revealing proliferative lymphatic channels, original magnification ×100).