scholarly journals Pulmonary Langerhans Cell Histiocytosis-associated Pulmonary Hypertension Showing a Drastic Improvement Following Smoking Cessation

2016 ◽  
Vol 55 (5) ◽  
pp. 491-495 ◽  
Author(s):  
Yoshiaki Kinoshita ◽  
Kentaro Watanabe ◽  
Atsuhiko Sakamoto ◽  
Kouko Hidaka
Keyword(s):  
Smoking Cessation ◽  
Pulmonary Hypertension ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Pulmonary Langerhans Cell Histiocytosis

scholarly journals Pulmonary Langerhans Cell Histiocytosis: An Update From the Pathologists' Perspective

2016 ◽  
Vol 140 (3) ◽  
pp. 230-240 ◽  
Author(s):  
Anja C. Roden ◽  
Eunhee S. Yi
Keyword(s):  
Pulmonary Hypertension ◽  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Additional Treatment ◽  
Langerhans Cell ◽  
Braf V600e ◽  
First Line ◽  
Clonal Proliferation ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Appropriate Treatment

Context Pulmonary Langerhans cell histiocytosis (PLCH) is a rare histiocytic disorder that almost exclusively affects the lungs of smokers. PLCH is characterized by bronchiolocentric nodules and/or cysts in an upper and mid lung distribution with sparing of the costophrenic angles. The diagnosis can be challenging and often requires transbronchial biopsy or surgical lung biopsy. Pulmonary hypertension is a relatively common and sometimes severe complication of PLCH. The pathogenesis of PLCH is still debated. Recently, BRAF V600E mutation and BRAF expression have been identified in some patients with PLCH, suggesting that at least a subset of PLCH has a clonal proliferation. While smoking cessation is the first-line treatment of PLCH, some patients might require additional treatment and eventually transplant. Given that the lesional cells of PLCH express BRAF in some patients, MAPKinase pathway–targeted treatment might be useful for therapy-resistant patients. Objective —To present the more recently recognized clinical and pathologic aspects of PLCH, including pulmonary hypertension in PLCH, pathogenesis, and treatment, as well as the basic diagnostic approach to PLCH. Data Sources Authors' own research, and search of literature database (PubMed) and UpToDate. Conclusions —Despite the recent progress, more studies are needed to elucidate the biology of PLCH for identification of prognostic factors and appropriate treatment options, especially for therapy-refractory PLCH cases.

scholarly journals Autopsy Findings in A Case of Pulmonary Langerhans Cell Histiocytosis-Associated Pulmonary Hypertension

2017 ◽  
Vol 18 ◽  
pp. 1401-1406
Author(s):  
Daisuke Taniyama ◽  
Hirofumi Kamata ◽  
Keisuke Miyamoto ◽  
Shuko Mashimo ◽  
Fumio Sakamaki
Keyword(s):  
Pulmonary Hypertension ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Autopsy Findings ◽  
Pulmonary Langerhans Cell Histiocytosis

Mechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis

CHEST Journal ◽  
2020 ◽  
Vol 158 (6) ◽  
pp. 2440-2448
Author(s):  
Glaucia Itamaro Heiden ◽  
Juliana Barbosa Sobral ◽  
Carolina Salim Gonçalves Freitas ◽  
André Luis Pereira de Albuquerque ◽  
João Marcos Salge ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Exercise Limitation ◽  
Pulmonary Langerhans Cell Histiocytosis

Echocardiographic and Clinical Characteristics of Pulmonary Hypertension Complicating Pulmonary Langerhans Cell Histiocytosis

2004 ◽  
Vol 79 (10) ◽  
pp. 1269-1275 ◽  
Author(s):  
Nithima Chaowalit ◽  
Patricia A. Pellikka ◽  
Paul A. Decker ◽  
Marie-Christine Aubry ◽  
Michael J. Krowka ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Characteristics ◽  
Langerhans Cell ◽  
Pulmonary Langerhans Cell Histiocytosis

scholarly journals A confusing case report of pulmonary langerhans cell histiocytosis and literature review

Open Medicine ◽  
2016 ◽  
Vol 11 (1) ◽  
pp. 178-182
Author(s):  
Yang Li ◽  
Wang Zhen ◽  
Ulrich Costable ◽  
Xu Jun ◽  
Ren Zhe ◽  
...  
Keyword(s):  
Smoking Cessation ◽  
Case Report ◽  
Literature Review ◽  
Rare Disease ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
First Line ◽  
Therapy Options ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Insidious Onset

AbstractPulmonary Langerhans Cell Histiocytosis (PLCH) is a rare disease. From the insidious onset and nonspecific manifestations, it is difficult to diagnose PLCH. To help improve the diagnosis and therapy options of adult PLCH, we present this case report and literature review about a confusing case of PLCH. In this report, we present a 37-year-old male PLCH case that was negative for CD1a and S100 expression. Smoking cessation and use of prescribed Spiriva appeared to improve the patient’s symptoms. To the best of our knowledge, this is the first reported case of PLCH in which improved symptoms were seen with the use of Spiriva alone.The mechanism is not clear, but potentially has some relationship with dilating the airway, decreasing the mucous hypersecretion and promoting anti-inflammatory pathways. From this patient’s case, we may be able to find more cases to then find other first line therapies for PLCH patients.

scholarly journals Smoking and Other Interstitial Lung Diseases

2011 ◽  
Vol 2011 ◽  
pp. 1-7
Author(s):  
Carpio Carlos ◽  
Gómez-Carrera Luis ◽  
Álvarez-Sala Rodolfo
Keyword(s):  
Computed Tomography ◽  
Smoking Cessation ◽  
Langerhans Cell Histiocytosis ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Langerhans Cell ◽  
Lung Disorder ◽  
High Resolution Computed Tomography ◽  
Pulmonary Langerhans Cell Histiocytosis

Cigarette smoking has been implicated in the development of some uncommon respiratory interstitial diseases. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung diseases are characterized by a diffuse alveolar and peribronchiolar filling with macrophages, respectively. Pulmonary Langerhans' cell histiocytosis is a rare interstitial lung disorder characterized by the proliferation of Langerhans' cell forming interstitial infiltrates and nodules that could progress to cavitary nodules. The treatment of these disorders involves smoking cessation and sometimes the use of steroids. High-resolution computed tomography is essential for the characterization of these smoking-related interstitial lung diseases, but frequently it is necessary to create a workgroup composed by pulmonologists, pathologists, and radiologists to diagnosis and treat patients affected with these pathologies.

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