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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Langerhans cell histiocytosis (LCH) is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but cor pulmonale may be observed in advanced disease. The chest radiograph is typically abnormal with nodular or interstitial infiltrates and cystic changes. High-resolution computed tomography of the chest with these findings in the middle and upper lobes of an adult smoker is virtually diagnostic of LCH. Pulmonary function assessment is variable. Asthma has rarely been reported in association with this disorder. There are only three reported cases of the diagnosis of concomitant asthma which have been made in association with the diagnosis of LCH. We present a case in which our patient presented with signs and symptoms of asthma to include confirmatory findings of airway hyperresponsiveness. The diagnosis of LCH was established after the patient failed to respond to conventional treatment for asthma, and further evaluation was completed.
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PMID:Langerhans cell histiocytosis presenting as uncontrolled asthma. 2406 95

A 28-year-old woman of Laotian origin presented with dry cough, fevers, night sweats and weight loss over the preceding 6 weeks. Radiological investigations revealed large complex cavitary lesions in the middle and lower lobes of the right lung, with no mediastinal lymph node enlargement. Bronchoscopic investigations did not yield a diagnosis. CT-guided fine-needle aspiration raised the possibility of Langerhans cell histiocytosis. Open lung biopsy initially supported this, but eventually a microscopic and immunohistochemical diagnosis of Hodgkin's lymphoma was made. The final diagnosis was primary pulmonary Hodgkin's lymphoma. We present a new case as well as a literature review of reported cases published since 2006.
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PMID:Primary pulmonary Hodgkin's lymphoma and a review of the literature since 2006. 2471 77

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.
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PMID:Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report. 2650 47

Langerhans cell histiocytosis (LCH) is a rare clonal disorder of unknown etiology and characterized by the proliferation of dendritic cells. LCH most commonly involves the bone followed by the skin and the lymph nodes. Recently, only a few cases of LCH with predominant lymph node involvement have been diagnosed by fine-needle aspiration cytology (FNAC). A 2-year-old boy presented with generalized lymphadenopathy, fever, and cough. The patient had hepatosplenomegaly, anemia, and lytic lesions in the skull. FNAC from the largest submandibular lymph node showed features of LCH. The large cells of LCH showed positive immunostaining for S-100 protein on FNAC smears. Later, lymph node biopsy and immunohistochemistry against S-100 protein and CD1a confirmed the diagnosis of LCH. The patient was treated with chemotherapy and he is under regular follow-up. This case report highlights the importance of FNAC as a rapid and accurate investigation in the diagnosis of lymph node predominant LCH.
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PMID:Langerhans cell histiocytosis diagnosed by FNAC of lymph nodes. 2688 3

Pulmonary arterial hypertension (PAH) secondary to pulmonary Langerhans cell histiocytosis (PLCH) is known to be a relatively common complication and is associated with a poor prognosis. However, the optimal therapeutic approach for these cases remains to be established. A 57-year-old man visited our hospital because of a progressive dry cough. A thoracic computed tomography examination showed a combination of diffuse thick-walled cysts and reticulonodular shadows that were predominant in bilateral upper lobes of the lungs. He was diagnosed as having PLCH based on the results of video-assisted thoracoscopic lung biopsies. During a 3-year clinical course, his condition deteriorated despite smoking cessation. A systemic evaluation demonstrated precapillary PAH caused by PLCH (PAH-PLCH), and treatment with tadalafil, a phosphodiesterase-5 inhibitor, was started. During a 50-month period of treatment with tadalafil, improvements in his dyspnea, 6-min walking distance, and hemodynamics were maintained without either overt hypoxemia or pulmonary edema. We considered that tadalafil therapy may be a useful option in the treatment of patients with PAH-PLCH.
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PMID:Long-term improvement during tadalafil therapy in a patient with pulmonary hypertension secondary to pulmonary Langerhans cell histiocytosis. 2733 Sep 52

Pulmonary Langerhans cell histiocytosis (PLCH) is a well known entity in adults but is exceedingly rare in children. It is better described in adults than in children. Smoking is a major etiological factor in adulthood. We report a case of a previously healthy 16-year-old male with a history of cigarette smoking, who presented with cough with sputum, breathlessness, easy fatigability and loss of appetite for two weeks. He was first diagnosed with bronchiectasis according to the cystic pulmonary changes demonstrated by computed tomography. After appropriate treatment, there was no sign of clinical improvement. A lung biopsy confirmed Langerhans cell histiocytosis (LCH). The definitive diagnosis was isolated pulmonary LCH. PLCH should be considered in the etiology of cystic lung diseases. Isolated pulmonary LCH is rare so such cases are needed to be reported and followed-up to understand the treatment response and course of this illness.
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PMID:Primary pulmonary Langerhans cell histiocytosis associated with smoking in an adolescent boy. 2974 22

Histiocytic disorders are a group of rare diseases with systemic involvement and with multiple clinical manifestations. We present the case of a 51 years old patient investigated for dyspnea with orthopnea, dry cough, asthenia, muscular weakness and ataxia. The association of previous symptoms with skin lesions, diabetes insipidus, partial hypophyseal insufficiency and pericarditis induced many diagnostic debates. The diagnosis is Histiocytosis X must be sustained by tissue biopsy with immunohistochemical assay or genetic testing. The particularity of our patient is the presence of pericarditis, rarely associated with histiocytosis. Collaboration between medical specialties is mandatory in order to treat this disorder.
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PMID:Histiocytosis X and Pericarditis - A Rare Association and a Difficult Diagnosis. 3058 98

Respiratory bronchiolitis-associated interstitial lung disease (ILD), desquamative interstitial pneumonia (DIP), and pulmonary Langerhans cell histiocytosis are entities of smoking-related ILD. While clinically regarded as 2 separate forms of idiopathic interstitial pneumonia, DIP, and respiratory bronchiolitis-associated ILD are thought to be representing ends of a continuous spectrum of disease that primarily affects tobacco smokers. This case report presents a 53-year-old female patient who has 58 pack-year smoking history who has been experiencing a dry cough and epigastric pains for 2 years. Open-lung biopsy is performed and histopathology indicated smoking-related interstitial fibrosis. The patient did not stop smoking, which after a year leads to significant clinical deterioration with a notable decrease in diffusion for carbon monoxide capacity. Upon smoking cessation and treatment with corticosteroids, a significant clinical improvement is achieved. In smokers complaining of cough and reduced exercise tolerance and in whom evidence of interstitial fibrosis is demonstrated radiologically, DIP should be considered as a differential diagnosis. Smoking is the exclusive etiologic factor of pathogenesis of DIP.
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PMID:Desquamative interstitial pneumonia with clinical, radiological and histologic correlation. 3081 49

A 30-year old man who smoked, presented with cough, dyspnoea and fatigue. Radiography showed diffuse bilateral symmetrical nodules, partially cavitating. The histopathological findings from the thoracoscopic wedge resection were specific for pulmonary Langerhans cell histiocytosis. The patient immediately quit smoking, which resulted in less complaints and decreased abnormalities on the x-ray.
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PMID:[A smoking, coughing young adult]. 3260 27


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