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Pulmonary Langerhans cell histiocytosis PLCH is proliferation of monoclonal Langerhans cells in lung interstitium and airspaces. Etiology is unknown, but cigarette smoking plays a primary role. Symptoms are dyspnea, cough, fatigue, and pleuritic chest pain.
Diagnosis is based on history and imaging tests and sometimes on bronchoalveolar lavage and biopsy findings.
Treatment is smoking cessation. Corticosteroids are given in many cases, but efficacy is unknown. Lung transplantation is usually curative when combined with smoking cessation. Patients are at increased risk of cancer.
Pulmonary Langerhans cell histiocytosis is a disease in which monoclonal CD1a-positive Langerhans cells a type of histiocyte infiltrate the bronchioles and alveolar interstitium, accompanied by lymphocytes, plasma cells, neutrophils, and eosinophils.
PLCH is one manifestation of Langerhans cell histiocytosis , which can affect organs in isolation most notably the lungs, skin, bones, pituitary, and lymph nodes or simultaneously.
The etiology of PLCH is unknown, but the disease occurs almost exclusively in whites 20 to 40 years of age who smoke.
Men and women are affected equally. Women develop disease later, but differences in age at onset by sex may represent differences in smoking behavior. Pathophysiology may involve recruitment and proliferation of Langerhans cells in response to cytokines and growth factors secreted by alveolar macrophages in response to cigarette smoke.
Typical symptoms and signs of pulmonary Langerhans cell histiocytosis are dyspnea, nonproductive cough, fatigue, fever, weight loss, and pleuritic chest pain. There are few signs of PLCH; the physical examination results are usually normal. Pulmonary Langerhans cell histiocytosis is suspected based on history and chest x-ray and is confirmed by HRCT and bronchoscopy with biopsy and bronchoalveolar lavage. Chest x-ray classically shows bilaterally symmetric nodular opacities in the middle and upper lung fields with cystic changes and normal or increased lung volumes.
The lung bases are often spared. Appearance may mimic chronic obstructive pulmonary disease COPD or lymphangioleiomyomatosis. Pulmonary function test findings are normal, restrictive, obstructive, or mixed depending on when the test is done during the course of the disease.
Most commonly, the diffusing capacity for carbon monoxide DLCO is reduced and exercise is impaired. Bronchoscopy and biopsy are indicated when imaging and pulmonary function tests are inconclusive. Biopsy shows proliferation of Langerhans cells with occasional clustering of eosinophils the origin of the outdated term eosinophilic granuloma in the midst of cellular and fibrotic nodules that may take on a stellate configuration.
However, some patients develop slowly progressive disease, for which the clinical markers include. Cause of death is respiratory insufficiency or cancer. Lung cancer risk is increased because of cigarette smoking. The main treatment of pulmonary Langerhans cell histiocytosis is smoking cessation , which leads to symptom resolution in up to one third of patients.
Empiric use of corticosteroids and cytotoxic drugs is common practice even though their effectiveness is unproved. Lung transplantation is an option for otherwise healthy patients with accelerating respiratory insufficiency, but the disorder may recur in the transplanted lung if the patient continues or resumes smoking. In pulmonary Langerhans cell histiocytosis PLCH , monoclonal Langerhans cells proliferate in alveolar interstitium and bronchioles. Consider PLCH in patients age 20 to 40 who smoke and in whom chest x-ray shows bilaterally symmetric nodular opacities in the middle and upper lung fields with cystic changes.
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More Content. Click here for Patient Education. Continued smoking. Smoking cessation. Possibly corticosteroids and cytotoxic drugs or lung transplantation. Confirm the diagnosis with high-resolution CT or, if results are inconclusive, lung biopsy. Consider corticosteroids and cytotoxic drugs and, if smoking has ceased, lung transplantation.
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Langerhans cell histiocytosis
Pulmonary Langerhans cell histiocytosis PLCH is proliferation of monoclonal Langerhans cells in lung interstitium and airspaces. Etiology is unknown, but cigarette smoking plays a primary role. Symptoms are dyspnea, cough, fatigue, and pleuritic chest pain. Diagnosis is based on history and imaging tests and sometimes on bronchoalveolar lavage and biopsy findings. Treatment is smoking cessation.
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Pulmonary Langerhans Cell Histiocytosis
This article focuses on the latter. PLCH is usually identified in young adults years of age. It is a rare disorder with no well-established gender predilection, which appears to be more common in Caucasian populations 4. Presentation is usually with dyspnea or a non-productive cough. Other symptoms include constitutional symptoms fatigue and weight loss , pleuritic chest pain, or spontaneous pneumothorax 1,4. Up to a quarter of patients are asymptomatic. It is postulated that as these cellular granulomas evolve, peripheral fibrosis forms resulting in traction on the central bronchiole which becomes cyst-like 3.