Clinical features and outcome of adult patients with pulmonary Langerhans cell histiocytosis

Abstract

Pulmonary Langerhans’ cell histiocytosis (PLCH) is a neoplastic disorder with strong inflammatory component. The clinical manifestations, features, and outcome of the PLCH vary widely, and clinical course of the disease is unpredictable. Material and methods: 124 adults (61 women and 63 men in age 15 to 69 years) with LCH, have been presented in our Department for the last 21 years. The median follow-up period was 146 months (range 3 to 338 months). Results: Isolated PLCH was diagnosed in 90(72%) cases, multisystem disease in 30(26%) patients. Two (1.6%) patients had multifocal bone disease, one (0.8%) had isolated mucosal, and one isolated bone lesion. Out of whole group only 6(5%) patients were nonsmokers. Incidentally the disease was diagnosed in 20% of patients. Pneumothorax as a first symptom of the disease was observed in 26% of patients. The most common findings in the pulmonary function tests were obstructive ventilatory defect (57%), and decreasing of transfer factor for carbon monoxide (80%). During the time of observation 70(56%) patients did not require immunosuppressive therapy. Only in 3(14%) patients steroid treatment was sufficient, other 19(86%) patients required chemotherapy. Chemotherapy with vinblastine, prednisone and mercaptopurine was administered in 11 patients, but only in 4 the regression with non-active disease was observed. Sixteen (13%) patients were treated successfully with cladribine, and till now no relapse was noticed. Conclusions: PLCH is a rare, cystic lung disease, affects mainly young adult smokers, without gender predominance; early diagnosis, smoking cessation, and adequate treatment are critical in its management.