scholarly journals Pulmonary Langerhans Cell Histiocytosis Presenting as a Case of Diffuse Cystic Lung Disease with Diabetes Insipidus and Primary Sclerosing Cholangitis

2020 ◽  
Author(s):  
R. Samannan ◽  
E.A. Lang
Keyword(s):  
Diabetes Insipidus ◽  
Lung Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Cystic Lung Disease

scholarly journals Paediatric pulmonary Langerhans cell histiocytosis

Breathe ◽  
2020 ◽  
Vol 16 (2) ◽  
pp. 200003
Author(s):  
Mhairi Barclay ◽  
Rebecca Devaney ◽  
Jayesh. M. Bhatt
Keyword(s):  
Respiratory Failure ◽  
Lung Disease ◽  
Langerhans Cell Histiocytosis ◽  
Multidisciplinary Treatment ◽  
Langerhans Cell ◽  
List Type ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Cystic Lung Disease ◽  
Management Of Complications

Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare diffuse cystic lung disease. Unlike pulmonary Langerhans cell histiocytosis (LCH) in adults, which is often seen as an isolated condition with smoking being a major risk factor, isolated pPLCH is vanishingly rare in children and it is most often a component of multisystem LCH. Diagnosis should be based on histological and immunophenotypic examination of affected tissue in addition to clinical and radiological features. It should be considered an important differential for diffuse cystic lung disease in paediatric patients. Recent progress in the biological understanding of the disease supports the classification of LCH as an inflammatory myeloid neoplasia. Chemotherapy and specific management of respiratory complications are the mainstays of treatment. The lungs are no longer considered a “risk organ” in LCH as pulmonary involvement is not associated with a worse prognosis than the involvement of other organs. Multidisciplinary treatment approaches are needed.Prognosis can be good but is adversely influenced by multisystem involvement, and complications such as pneumothoraces and respiratory failure can be life threatening. This review aims to give an overview of this condition, with a focus on the diagnosis, monitoring and management of complications such as pneumothoraces and respiratory failure, which can be challenging for the paediatric respiratory specialist.Educational aimsTo give an overview of paediatric pulmonary LCH.To discuss the differential diagnosis of paediatric cystic lung disease.

Cystic Lung Disease

Chest Imaging ◽  
2019 ◽  
pp. 429-433
Author(s):  
Juliana Bueno
Keyword(s):  
Lung Disease ◽  
Langerhans Cell Histiocytosis ◽  
Normal Lung ◽  
Lung Cysts ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Cystic Lung Disease ◽  
Lymphocytic Interstitial Pneumonia ◽  
Cavitary Disease ◽  
Diffuse Lung

A lung cyst is a round parenchymal lucency or low-attenuating area with a well-defined interface with adjacent normal lung and surrounded by an epithelial or fibrous wall that is often less than 2 mm in thickness. Diseases that manifest with lung cysts comprise a limited but well known group of entities. Classic diseases manifesting with diffuse lung cysts include pulmonary Langerhans cell histiocytosis (PLCH), lymphangioleiomyomatosis (LAM), lymphocytic interstitial pneumonia (LIP) and Birt-Hogg-Dubé syndrome. However, it is important to recognize other conditions that may mimic cystic lung disease, such as pneumatoceles, tracheobronchial papillomatosis, cavitary disease (e.g. tuberculosis, fungal infection), bronchiectasis, and emphysema. Differentiation between true cysts and cyst mimics may be difficult, and may rely on identification of ancillary findings.

Pulmonary Langerhans Cell Histiocytosis

2020 ◽  
Vol 41 (02) ◽  
pp. 269-279
Author(s):  
Brian Shaw ◽  
Michael Borchers ◽  
Dani Zander ◽  
Nishant Gupta
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Mitogen Activated Protein Kinase ◽  
Myeloid Neoplasm ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Mitogen Activated Protein ◽  
The Central Nervous System

AbstractPulmonary Langerhans cell histiocytosis (PLCH) is a diffuse cystic lung disease that is strongly associated with exposure to cigarette smoke. Recently, activating pathogenic mutations in the mitogen-activated protein kinase pathway have been described in the dendritic cells in patients with PLCH and have firmly established PLCH to be an inflammatory myeloid neoplasm. Disease course and prognosis in PLCH are highly variable among individual patients, ranging from spontaneous resolution to development of pulmonary hypertension and progression to terminal respiratory failure. A subset of patients with PLCH may have extrapulmonary involvement, typically involving the skeletal system in the form of lytic lesions, skin lesions, or the central nervous system most commonly manifesting in the form of diabetes insipidus. Smoking cessation is the cornerstone of treatment in patients with PLCH and can lead to disease regression or stabilization in a substantial proportion of patients. Further insight into the underlying molecular pathogenesis of PLCH has paved the way for the future development of disease-specific biomarkers and targeted treatment options directed against the central disease-driving mutations.

scholarly journals Pulmonary Langerhans cell histiocytosis with inguinal abscess: Unusual presentation

2020 ◽  
Vol 29 (1) ◽  
pp. 64-66
Author(s):  
Kamarul Naim Mohd Hirmizi ◽  
Nurul Yaqeen Mohd Esa ◽  
Mardiana Abdul Aziz ◽  
Nor Salmah Bakar ◽  
Mohammad Hanafiah
Keyword(s):  
Lymph Node ◽  
Langerhans Cell Histiocytosis ◽  
Respiratory Symptoms ◽  
Inguinal Lymph Node ◽  
Langerhans Cell ◽  
Histopathological Analysis ◽  
Intermittent Fever ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
History Of

We report a case of a 30-year-old man who was treated as recurrent right inguinal abscess following a 2-month history of right inguinal swelling and intermittent fever with no respiratory symptoms. Resection of his right inguinal lymph node and the histopathological analysis revealed the diagnosis of Langerhans cell histiocytosis. In addition, the CT of the thorax showed presence of bilateral cystic lung changes consistent with pulmonary Langerhans cell histiocytosis.

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