scholarly journals Secondary pulmonary hypertension due to pulmonary Langerhans cell histiocytosis accompanied with panhypopituitarism

2020 ◽  
Vol 9 (1) ◽  
Author(s):  
Tatsuyuki Miyashita ◽  
Susumu Yamazaki ◽  
Hiromitsu Ohta ◽  
Hidetoshi Nakamura ◽  
Makoto Nagata
Keyword(s):  
Pulmonary Hypertension ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Secondary Pulmonary Hypertension

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

Pulmonary Langerhans Cell Histiocytosis-Associated Pulmonary Hypertension

CHEST Journal ◽  
2012 ◽  
Vol 142 (5) ◽  
pp. 1150-1157 ◽  
Author(s):  
Jérôme Le Pavec ◽  
Gwenaël Lorillon ◽  
Xavier Jaïs ◽  
Colas Tcherakian ◽  
Séverine Feuillet ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Pulmonary Langerhans Cell Histiocytosis
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