Abstract

           Lymphangioleiomyomatosis (LAM) is a rare disorder of unknown cause that affects women of reproductive age. Acute respiratory failure that is secondary to diffuse alveolar hemorrhage is an extremely rare manifestation in LAM patients.  In the present work, we describe a patient with a diffuse alveolar hemorrhage that led to acute respiratory failure and death.

A 29-year-old woman was hospitalized with progressive dyspnea.  Since March, there have been episodes of hemoptysis, intermittent chest pain. January 5, the condition deteriorated sharply.Was urgently hospitalized in the ICU. The patient progressed to acute respiratory failure and rapidly deteriorated with hypoxia. Mechanical ventilation with positive end-expiratory pressure was necessary.Thoracic computed tomography demonstrated multiple well-contoured round thin-walled cysts, bilateral pneumothorax, pneumomediastinum and emphysema of the soft tissues, zones of consolidation of the lung tissue due to hemorrhagic impregnation (fig.1).A bronchoscopic examination revealed a moderate amount of blood in the segments of both lungs. The patient developed hemodynamic instability that resulted in the patient's death. A histological examination during the autopsy revealed cystic lesions in the pulmonary tissue, with alveolar hemorrhaging and atypical smooth muscle cell proliferation at the cyst wall (fig.2). 

Acute respiratory failure that is secondary to diffuse alveolar hemorrhage is a very rare manifestation in LAM patients.To our knowledge, the present paper is the second report of an LAM patient presenting with diffuse pulmonary hemorrhage with a histological examination.