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.

scholarly journals The etiology of diffuse cystic lung diseases: an analysis of 1010 consecutive cases in a LAM clinic

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Han Cui ◽  
Chongsheng Cheng ◽  
Wenshuai Xu ◽  
Xinlun Tian ◽  
Yanli Yang ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Langerhans Cell Histiocytosis ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Langerhans Cell ◽  
Light Chain Deposition Disease ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Sjögren‘S Syndrome

Abstract Background The differential diagnosis of diffuse cystic lung disease (DCLD) is a clinical challenge. We wish to analyze the distribution of the etiology of DCLD based on data from a single lymphangioleiomyomatosis (LAM) clinic. Methods All DCLD patients at the LAM Clinic of Peking Union Medical College Hospital between January 2006 and December 2019 were analyzed. Information on the demographic, clinical, radiological, and pathological features was collected. Results A total of 1010 patients with DCLD on CT scan were evaluated. A sum of 711(70.4%) patients were diagnosed with definite or probable LAM. Other diagnoses included Birt–Hogg–Dubé syndrome (46), Sjogren's syndrome (38), pulmonary Langerhans cell histiocytosis (14), lung tumors (3), Castleman disease (2), antineutrophil cytoplasmic antibody-associated vasculitis (2), systemic lupus erythematosus (1), Marfan syndrome (1), amyloidosis (1), congenital cystic adenomatoid malformation of the lung (1), and pleuroparenchymal fibroelastosis (1). In the 38 patients diagnosed with Sjogren's syndrome, 2 were diagnosed with light-chain deposition disease, 2 were diagnosed with amyloidosis and 1 was diagnosed with lymphocytic interstitial pneumonia. One hundred and eighty-nine patients (18.7%) were undiagnosed. Lung biopsy results were available in 27 patients in the undiagnosed DCLD group but did not provide a diagnosis. Conclusion Approximately 70% of DCLD patients in our LAM clinic had LAM. The common differential diagnoses included Birt–Hogg–Dubé syndrome, Sjogren’s syndrome, and pulmonary Langerhans cell histiocytosis. Detailed clinical information and laboratory, genetic, and pathological investigations provide correct diagnoses in most patients with DCLD.

scholarly journals Multiple cystic lung disease

2015 ◽  
Vol 24 (138) ◽  
pp. 552-564 ◽  
Author(s):  
Flavia Angélica Ferreira Francisco ◽  
Arthur Soares Souza ◽  
Gláucia Zanetti ◽  
Edson Marchiori
Keyword(s):  
Lung Disease ◽  
Open Lung Biopsy ◽  
Diagnostic Challenge ◽  
High Resolution Computed Tomography ◽  
Disease Evolution ◽  
Lung Cysts ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Cystic Lung Disease ◽  
Cystic Metastasis

Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and accurate diagnosis of the majority of these diseases without recourse to open-lung biopsy. The main diseases in this group that are discussed in this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and folliculin gene-associated syndrome (Birt–Hogg–Dubé); other rare causes of cystic lung disease, including cystic metastasis of sarcoma, are also discussed. Disease progression is unpredictable, and understanding of the complications of cystic lung disease and their appearance during evolution of the disease are essential for management. Correlation of disease evolution and clinical context with chest imaging findings provides important clues for defining the underlying nature of cystic lung disease, and guides diagnostic evaluation and management.

scholarly journals Disturbances of pulmonary microcirculation in patients with pulmonary Langerhans' cell histiocytosis with different changes of pulmonary function tests

2016 ◽  
Vol 15 (3) ◽  
pp. 30-35 ◽  
Author(s):  
V. P. Zolotnitskaya ◽  
V. I. Amosov ◽  
D. V. Dsadsua
Keyword(s):  
Computed Tomography ◽  
Respiratory Failure ◽  
Langerhans Cell Histiocytosis ◽  
Pulmonary Function Tests ◽  
Langerhans Cell ◽  
Histiocytosis X ◽  
Emission Computed Tomography ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Stage Of Development ◽  
Pulmonary Microcirculation

Introduction and purpose. Disturbances of pulmonary microcirculation in patients with pulmonary Langerhans' cell histiocytosis and various types of respiratory failure are characterized by different scintigraphic patterns. Aim: to identify features of the circulatory disturbances in the lungs of patients with histiocytosis X, and various forms of bronchial conductivity changes. Material and methods: analysis of changes of microcirculation in the lungs in 54 patients with histiocytosis X, with different types of respiratory insufficiency, assessed by means of single photon emission computed tomography, perfusion scintigraphy and multislice computed tomography. Results and conclusions: in patients with histiocytosis x with obstructive type of respiratory failure, microcirculatory changes were detected at a very early stage of development of the disease. In the terminal stage of the disease, a typical scintigraphic pattern «candle flame» was observed. In patients with combined restrictive and obstructive changes (mixed type), no differences from changes of blood flow in obstructive variant were observed. In the restrictive type of respiratory failure, disorders of microcirculation do not have any typical scintigraphic pattern. The presence of characteristic nodular lesions and increasing fibrotic changes of all structural units were observed in the parenchyma of the lung.

scholarly journals Clarifying the relationship between pulmonary langerhans cell histiocytosis and Alpha 1 antitrypsin deficiency

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Cormac McCarthy ◽  
Emmanuelle Bugnet ◽  
Amira Benattia ◽  
Michael P. Keane ◽  
Benoit Vedie ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Entire Study ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Study Population ◽  
Alpha 1 Antitrypsin ◽  
The Relationship

AbstractPulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking related, progressive diffuse cystic lung disease that occurs primarily in smokers. The aim of this study was to determine if there was an increase in alpha-1 antitrypsin deficient alleles or phenotypes in a large series of PLCH patients and whether serum alpha-1 antitrypsin levels correlated with markers of disease severity. Fifty PLCH patients, 24 with a diffuse cystic lung pattern and 26 with a typical nodulo-cystic pattern on imaging were included. The mean alpha-1 antitrypsin levels were in normal range for both the population with diffuse cystic lung pattern population (1.39 g/L ± 0.37) and the nodulo-cystic pattern group (1.41 g/L ± 0.21). Deficiency alleles PiZ and PiS were 1% and 2% respectively in the entire study population of 50 patients, demonstrating no increased incidence of alpha-1 antitrypsin deficiency in PLCH. Alpha-1 antitrypsin levels showed no correlation with lung function parameters or extent of cystic lesions on lung computed tomography.

Sign in / Sign up

Export Citation Format

Share Document