Open AccessLymphangioleiomyomatosis and Langerhans cell histiocytosis - two case reports from our practice
Author(s) -
Ana Milenković,
Miroslav Ilić,
Jovan Javorac,
Nataša Savić,
Nensi Lalić
Publication year - 2022
Publication title -
srpski arhiv za celokupno lekarstvo
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.135
H-Index - 17
eISSN - 2406-0895
pISSN - 0370-8179
DOI - 10.2298/sarh211203034m
Subject(s) - lymphangioleiomyomatosis , medicine , tuberous sclerosis , langerhans cell histiocytosis , asymptomatic , histiocytosis , lung , lung biopsy , pulmonary function testing , pediatrics , radiology , biopsy , pathology , disease
. Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) 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 LAM (43 y.) has pulmonary changes detected during regular examination within the underlying disease - tuberous sclerosis. Four years after starting therapy with everolimus she is still respiratory asymptomatic, a slight radiological deterioration of cystic changes is registered, the diffusion capacity is declining (for 12%). The second patient (23 y.) 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 has been concluded that it is Langerhans cell histiocytosis. Cessation of smoking was advised. The follow-up examinations show withdrawal of symptoms and an orderly finding of lung function, chest HRCT indicates slight regression of changes. In the meantime, the patient gave birth to a healthy child, the pregnancy and prenatal period went smoothly. Conclusion. These diseases are extremely rare and in cooperation with other specialties should be distinguished from diseases that mimic lung cysts.