Pulmonary Langerhans' Cell Histiocytosis in Adults

Abstract

Pulmonary Langerhans' cell histiocytosis (PLCH) is a rare disorder of unknown cause characterised by the infiltration of the lungs and other organs by the bone marrow derived Langerhans' cells, which carry mutations of BRAF gene and/or NRAS, KRAS and MAP2K1 genes. It occurs predominantly in young smokers, without gender predominance. The disease is characterised by formation of eosinophilic granulomas with the presence of Langerhans' cells infiltrating and destroying distal airways. High-resolution computed tomography of the chest (HRCT) plays an outstanding role in PLCH diagnosis. The typical radiological picture of PLCH is the presence of small intralobular nodules, often forming 'tree in bud' lesions, cavitated nodules, thin-and thick-walled cystic lesions frequently confluent. Definite diagnosis requires the finding of characteristic lesions in histological examination and demonstration of antigen CD1a or CD207 presenting cells in immunohistochemistry. Smoking cessation is the most important recommendation for PLCH patients. There are no evidence based data regarding systemic steroid therapy. The treatment of progressive PLCH is based on cladribine or cytarabine as salvage therapy. The prognosis is good, and over 85% of patients survive 10 years.

Pneumothorax in Patients with Pulmonary Langerhans Cell Histiocytosis

“…LCH lesions can be observed in multiple organs or a single organ, usually the skin, bone, lymph nodes, or lungs. Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related disease characterised by the development of nodular and cystic lung lesions that predispose to pneumothorax [3–7]. It is a disease of young and middle-aged people, mainly smokers, and affects both sexes equally.…”

“…It is a disease of young and middle-aged people, mainly smokers, and affects both sexes equally. The course of LCH is unpredictable, and ranges from spontaneous regression (particularly in adults with pulmonary involvement who quit smoking) and stabilisation with or without relapses to continuous progression despite aggressive chemotherapy [3–7]. …”

Pneumothorax in young non-smoking patient

“…Diseases that can cause secondary pneumothorax include chronic obstructive pulmonary disease, emphysema, lung abscess, tuberculosis, pneumonia, lymphangioleiomyomatosis, lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, alpha -1 antitrypsin deficiency, or pulmonary LCH (PLCH). [1][2][3][4][5] PLCH is a rare disease, which usually affects young people and smokers. LCH may be limited to the lungs, or the lungs may be one of the involved organs, both at the time of diagnosis and during follow -up.…”

Cystic Lung Disease in an Adult Non-Smoker

“…Steroids have been shown to be beneficial in pLCH, especially in isolated pulmonary involvement in adults. Cladribine has also been tried in non-responsive cases [6]. Cases of pneumothorax are treated with standard measures which include, among others, intercostal drainage via tubes with pleurodesis.…”

“…The patient has been counseled for the future need of a lung transplant. Five-year survival has been reported to be more than 50%, but post-transplant recurrence has also been shown in 20% of patients [6].…”