Abstract
BackgroundThe study aimed to investigate the clinical features and prognosis factors of adult patients with Langerhans cell histiocytosis (LCH) with pulmonary involvement, especially multisystem (MS) LCH with pulmonary involvement.MethodsWe retrospectively analyzed the demographic materials, clinical features and treatment outcomes of 119 adult LCH patients with pulmonary involvement at our center from January 1990 to November 2019.ResultsAmong 119 patients, 13 (10.9%) had single-system (SS) LCH, and 106 (89.1%) had MS-LCH with pulmonary involvement. SS-LCH patients had higher smoking rate (84.6% vs 52.8%, P = 0.026) and smoking index (300 vs 200, P = 0.019) than MS-LCH patients. The percentage of respiratory symptoms of SS-LCH patients was higher than MS-LCH patients (84.6% vs 53.8%, P = 0.034). Pulmonary function was impaired in 83.8% of the patients, and DLCO was the parameter most frequently impaired, accounting for 81.1%. The median DLCO was 65.1% predicted. Patients with pneumothorax had significantly worse DLCO (P = 0.022), FEV1 (P = 0.000) and FEV1/FVC (P = 0.000) than those without pneumothorax. During the follow-up, 72.4% of the patients had stable pulmonary function, and 13.8% showed improvements after chemotherapy. The estimated 3-year OS and EFS were 89.7 and 58.3%, respectively. Patients with a baseline FEV1 ≤ 55% predicted had worse OS. A history of pneumothorax indicated worse EFS and cytarabine based therapy predicted better EFS.ConclusionsAn FEV1 ≤ 55% predicted and a history of pneumothorax at diagnosis indicated a poor prognosis. Cytarabine based regimen may arrest the decline in pulmonary function in LCH patients with pulmonary involvement and improve EFS.
Highlights
The study aimed to investigate the clinical features and prognosis factors of adult patients with Langerhans cell histiocytosis (LCH) with pulmonary involvement, especially multisystem (MS) LCH with pulmonary involvement
We retrospectively reviewed the medical records of adult patients with LCH with pulmonary involvement who were evaluated at our center over a 30-year period
Patients There were 237 adult LCH patients diagnosed in Peking Union Medical College Hospital between January 1990 and November 2019 in total
Summary
The study aimed to investigate the clinical features and prognosis factors of adult patients with Langerhans cell histiocytosis (LCH) with pulmonary involvement, especially multisystem (MS) LCH with pulmonary involvement. Previous studies have mostly described the features of solitary lung lesions, while pulmonary involvement in MS-LCH has been less described, limiting our understanding of the overall perspective of the disease. Few studies have investigated the outcomes and prognosis of PLCH. The study conducted by the Histiocyte Society Adult Registry reported that LCH patients with isolated pulmonary lesions had much lower survival rates than those who had MS-LCH with lung involvement [8]. As a result, describing the clinical features, treatment outcomes and prognoses of adult LCH patients with pulmonary involvement is necessary to help people understand this rare disease
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