ABSTRACT
OBJECTIVES@#Pulmonary Langerhans cell histiocytosis (PLCH) is a clonal disease, characterized by proliferation of Langerhans cells that derived from bone marrow infiltrating the lungs and other organs. Due to the rarity of the disease, the current understanding of the disease is insufficient, often misdiagnosed or missed diagnosis. This study aims to raise clinicians' awareness for this disease via summarizing the clinical characteristics, imaging features, and treatment of PLCH.@*METHODS@#We retrospectively analyzed clinical and follow-up data of 15 hospitalized cases of PLCH from September 2012 to June 2021 in the Second Xiangya Hospital of Central South University.@*RESULTS@#The age of 15 patients (9 men and 6 women, with a sex ratio of 3 to 2) was 21-52 (median 33) years. Among them, 8 had a history of smoking and 5 suffered spontaneous pneumothorax during disease course. There were 3 patients with single system PLCH and 12 patients with multi-system PLCH, including 7 patients with pituitary involvement, 7 patients with lymph node involvement, 6 patients with bone involvement, 5 patients with liver involvement, 2 patients with skin involvement, 2 patients with thyroid involvement, and 1 patients with thymus involvement. The clinical manifestations were varied but non-specific. Respiratory symptoms mainly included dry cough, sputum expectoration, chest pain, etc. Constitutional symptoms included fever and weight loss. Patients with multi-system involvement experienced symptoms such as polyuria-polydipsia, bone pain, and skin rash. All patients were confirmed by pathology, including 6 by lung biopsy, 3 by bone biopsy, 2 by lymph node biopsy, and 4 by liver, skin, suprasternal fossa tumor, or pituitary stalk biopsy. The most common CT findings from this cohort of patients were nodules and/or cysts and nodular and cystic shadows were found in 7 patients. Three patients presented simple multiple cystic shadows, 3 patients presented multiple nodules, and 2 patients presented with single nodules and mass shadows. Pulmonary function tests were performed in 4 patients, ventilation dysfunction was showed in 2 patients at the first visit. Pulmonary diffusion function tests were performed in 4 patients and showed a decrease in 3 patients. Smoking cessation was recommended to PLCH patients with smoking history. Ten patients received chemotherapy while 2 patients received oral glucocorticoid therapy. Among the 11 patients with the long-term follow-up, 9 were in stable condition.@*CONCLUSIONS@#PLCH is a neoplastic disease closely related to smoking. The clinical manifestations and laboratory examination are not specific. Pneumothorax could be the first symptom which is very suggestive of the disease. Definitive diagnosis relies on histology. There is no unified treatment plan for PLCH, and individualized treatment should be carried out according to organ involvement. Early smoking cessation is essential. Chemotherapy is the main treatment for rapidly progressing PLCH involved multiple organs. All diagnosed patients can be considered for the detection of BRAFV600E gene and relevant targeted therapies have been implemented recently.
Subject(s)
Adult , Female , Humans , Male , Cysts , Histiocytosis, Langerhans-Cell/therapy , Lung/pathology , Retrospective Studies , Smoking/adverse effects , Smoking CessationABSTRACT
Background: Smoking induced lung diseases constitute a complex group of disorders, varying from the well-known entity of Chronic Obstructive Pulmonary Disease (COPD) to the more recently described interstitial lung diseases. Aim and objectives: The aim of the study was to delineate the relation between cigarette smoking and the development of interstitial lung disease with declaration of the different types of the interstitial lung associated with smoking. Materials and methods: This was prospective study consisting of patients with smoking history referred to Department of Radiodiagnosis of NRIGH for HRCT with symptoms of progressive dyspnea. Inclusion criteria were smokers with progressive dyspnea, (age range 30-90 years, mean age 54 years ± 8). HRCT was done to all subjects using GE Light Speed Multislice 4 channels. CT scan examination was performed using GE Light Speed Multislice 4 channels present in our radiology department. The examination was done in supine position. A scout was taken with kV 120 and mA 120, then helical scanning was done in caudo-cranial direction to minimize respiration artifacts, using detector row 4, helical thickness 1.25, pitch 1.5:1, speed (mm/rot) 7.5, Detector configuration 4 · 1.25, beam collimation 5.00 mm, interval 1.00, gantry tilt 0.0, FOV depends on the patients’ body build, but is about 35 cm, kV 120–140, mA 120–160, total exposure time about 16 s during breath hold in inspiration. The images acquired were sent to a separate workstation to be processed, manipulated and reconstructed. Reconstruction of the images was done using reconstruction software available at the workstation to attain HRCT axial, coronal and sagittal images. Also mediastinal window images were done for each case. Results: In the present study, the most common seen HRCT pattern was Idiopathic Pulmonary Fibrosis, most affected gender were males and most affected age group was between 60-70 years. Sreedevi Meka, D Ankamma Rao. Role of HRCT in Smoking Related Interstitial Lung Diseases. IAIM, 2019; 6(8): 78-94. Page 79 Conclusion: In the appropriate clinical evaluation and in the presence of typical findings, high - resolution CT plays an essential role in evaluation and definite diagnosis of Smoking Related – Interstitial Lung Disease, and this may obviate further testing. However, lung biopsy may be needed when the high-resolution CT are relatively non-specific or when a confident definitive diagnosis is needed.
ABSTRACT
La histiocitosis de células de Langerhans pulmonar es una patología intersticial en la que existe un acumulo de células histiocíticas específicas a nivel pulmonar. El neumotórax espontáneo es una complicación reconocida de histiocitosis de células de Langerhans pulmonar y es secundario a la destrucción del parénquima pulmonar con cambios quísticos asociados. Reportamos el caso de un niño de 2 años con neumotórax espontáneo bilateral recurrente, con una tomografía axial computada de tórax con infiltrado intersticial, fibrosis, lesiones quísticas e imágenes bullosas. El diagnóstico fue establecido por examen histológico e inmunohistoquímica de tejido de biopsia pulmonar con anticuerpos CD1 y S100 positivos. El niño recibió tratamiento con prednisona y etopósido, con buena respuesta clínica y tomográfica.
Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.
Subject(s)
Child, Preschool , Humans , Male , Histiocytosis, Langerhans-Cell/complications , Pneumothorax/etiology , Histiocytosis, Langerhans-Cell/diagnosis , Pneumothorax/pathologyABSTRACT
A 41 year-old man had suffered from cough, sputum and febrile sense for several months. On the chest CT scan, small nodules, cystic air spaces, and pneumothorax were observed. Pulmonary Langerhans cell histiocytosis was confirmed by lung biopsy. Pulmonary Langerhans cell histiocytosis in adult is an uncommon granulomatous disease of unknown cause, which affects the lung, either in isolation or in addition to other organ systems. The pulmonary symptoms at the time of presentation were nonspecific, and symptomes by the involvement of other organs ocurr sometimes. Pneumothoraces commonly were complicated the course of pulmonary Langerhans cell histiocytosis and frequently recurred. We report a pulmonary Langerhans cell histiocytosis with pneumothorax in adult.