Clinical features and outcome of adult patients with pulmonary Langerhans cell histiocytosis

Radzikowska, Elżbieta; Wiatr, Elżbieta; Błasińska‐Przerwa, Katarzyna; Jeśkiewicz, Maria; R, Langfort; Maksymiuk, Beata; Modrzewska, Katarzyna; Bestry, Iwona; Załęska, Monika; Szopiński, Janusz; Jerzemska, Agnieszka; Ochman, Marek; Naumnik, Wojciech; Jastrzębski, Dariusz; Piotrowski, Wojciech J.; Roszkowski‐Śliż, Kazimierz

doi:10.1183/13993003.congress-2019.pa3683

Cited by 2 publications

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“…Similarly, only three (38%) out of eight patients with PLCH presented by Baqir et al ( 41 ) and 10 (25%) out of 40 patients presented by Elia et al ( 11 ) in whom BAL was performed displayed the presence of CD1a-positive cells over 5%. In our group of 38 patients with PLCH, only in eight (21%) did BAL assessment have a diagnostic value ( 47 ).…”

Section: Clinical Presentationmentioning

confidence: 75%

Update on Pulmonary Langerhans Cell Histiocytosis

Radzikowska¹

2021

Front. Med.

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Pulmonary Langerhans cell (LC) histiocytosis (PLCH) has unknown cause and is a rare neoplastic disorder characterized by the infiltration of lungs and various organs by bone marrow-derived Langerhans cells with an accompanying strong inflammatory response. These cells carry somatic mutations of BRAF gene and/or NRAS, KRAS, and MAP2K1 genes, which cause activation of the mitogen-activated protein kinase (MAPK)/extracellular signal-regulated kinase (ERK) signaling pathway. PLCH occurs predominantly in young smokers, without gender predominance. Lungs might be involved as an isolated organ or as part of a multiorgan disease. High-resolution computed chest tomography plays an outstanding role in PLCH diagnosis. The typical radiological picture of PLCH is the presence of small intralobular nodules, “tree in bud” opacities, cavitated nodules, and thin- and thick-walled cysts, frequently confluent. Histological examination of the lesion and demonstration of characteristic eosinophilic granulomas with the presence of LCs that display antigen CD1a or CD207 in immunohistochemistry are required for definite diagnosis. Smoking cessation is the most important recommendation for PLCH patients, but treatment of progressive PLCH and multisystem disease is based on chemotherapy. Recently, new targeted therapies have been implemented.

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Section: Clinical Presentationmentioning

confidence: 75%

Update on Pulmonary Langerhans Cell Histiocytosis

Radzikowska¹

2021

Front. Med.

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“…No association was identified between the presence of this mutation and disease presentation or outcome, in contrast to what has been reported in paediatric LCH populations who have higher risk of organ involvement. Two large retrospective studies, one from Russia and one from Poland, found a lack of response to corticosteroid therapy in LCH; conversely, cladribine was reported to be associated with 88-100% stabilisation/regression of the disease [69,70].…”

Section: Rare Ilds/dpldsmentioning

confidence: 99%

ERS International Congress, Madrid, 2019: highlights from the Interstitial Lung Diseases Assembly

et al. 2020

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This article discusses a selection of the scientific presentations in the field of interstitial lung diseases (ILDs) that took place at the 2019 European Respiratory Society International Congress in Madrid, Spain. There were sessions from all four groups within Assembly 12: group 12.01 “Idiopathic interstitial pneumonias”, group 12.02 “ILDs/diffuse parenchymal lung diseases (DPLDs) of known origin”, group 12.03 “Sarcoidosis and other granulomatous ILDs/DPLDs” and group 12.04 “Rare ILDs/DPLDs”. The presented studies brought cutting-edge developments on several aspects of these conditions, including pathogenesis, diagnosis and treatment. As many of the ILDs are individually rare, the sharing of experiences and new data that occur during the Congress are very important for physicians interested in ILDs and ILD patients alike.

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Clinical features and outcome of adult patients with pulmonary Langerhans cell histiocytosis

Cited by 2 publications

References 0 publications

Update on Pulmonary Langerhans Cell Histiocytosis

Update on Pulmonary Langerhans Cell Histiocytosis

ERS International Congress, Madrid, 2019: highlights from the Interstitial Lung Diseases Assembly

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