Cladribine improves lung cysts and pulmonary function in a child with histiocytosis

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Cladribine improves lung cysts and pulmonary function in a child with histiocytosis.

Langerhans’ cell histiocytosis (LCH) is a rare disease of unknown aetiology involving accumulation of Langerhans’ cell histiocytosis, organised in granuloma, in various organs [1]. Pulmonary LCH (PLCH), is characterised by focal Langerhans’ cell granulomas infiltrating and destroying distal bronchioles, which results in cysts, major destruction of the pulmonary tissue and pneumothorax [2, 3]. P...

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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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ژورنال

عنوان ژورنال: European Respiratory Journal

سال: 2014

ISSN: 0903-1936,1399-3003

DOI: 10.1183/09031936.00146114