Lymphangioleiomyomatosis and Langerhans cell histiocytosis - two case reports from our practice

Abstract

Introduction. Lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are the most common pulmonary cystic diseases. Although they differ in pathogenesis, they share several features. The aim of this paper is to present the similarities and differences between these diseases, as well as to describe two cases from our practice. Outlines of cases. The patient with lymphangioleiomyomatosis (43 years old) had pulmonary changes detected during a regular examination within the underlying disease – tuberous sclerosis. Four years after starting therapy with everolimus, she was still respiratory asymptomatic, a slight radiological deterioration of cystic changes was registered, the diffusion capacity was declining (by 12%). The second patient (23 years old) was admitted due to bilateral radiological lung changes and symptoms in the form of dry cough, quick fatigue, and chest pain. Pathohistological examination of the transbronchial biopsy showed numerous large-core histiocytes, immunohistochemically positive for CD1a and S100, so it was concluded that it was Langerhans cell histiocytosis. Cessation of smoking was advised. The follow-up examinations showed withdrawal of symptoms and an orderly finding of lung function, chest high-resolution computed tomography indicated slight regression of changes. In the meantime, the patient gave birth to a healthy child, the pregnancy and prenatal period were uneventful. Conclusion. These diseases are extremely rare and in cooperation with other specialties should be distinguished from diseases that mimic lung cysts.

Keywords

• lymphangioleiomyomatosis
• pulmonary langerhans cell histiocytosis
• cystic lung disease