Effect of cladribine therapy on lung cysts in pulmonary Langerhans cell histiocytosis

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Effect of cladribine therapy on lung cysts in pulmonary Langerhans cell histiocytosis

Cladribine therapy may be beneficial in advanced forms of pulmonary Langerhans cell histiocytosis, even that with multiple cystic changes http://ow.ly/yeLr30i0Tt6.

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Langerhans-cell histiocytosis (LCH) results from the accumulation of tissue histiocytes derived from the same progenitor cells as monocytes. Because cladribine is potently toxic to monocytes, we conducted a phase II trial of cladribine. Cladribine was administered to 13 LCH patients at 0.14 mg/kg per day by 2-hour intravenous infusion for 5 consecutive days, every 4 weeks for a maximum of six c...

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Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis

BACKGROUND Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterised by granulomatous proliferation of CD1a-positive histiocytes forming granulomas within lung parenchyma, in strong association with tobacco smoking, and which may result in chronic respiratory failure. Smoking cessation is considered to be critical in management, but has variable effects on outcome. No drug...

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ژورنال

عنوان ژورنال: ERJ Open Research

سال: 2018

ISSN: 2312-0541

DOI: 10.1183/23120541.00089-2017