Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis
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چکیده
منابع مشابه
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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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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Cladribine therapy may be beneficial in advanced forms of pulmonary Langerhans cell histiocytosis, even that with multiple cystic changes http://ow.ly/yeLr30i0Tt6.
متن کاملPulmonary langerhans cell histiocytosis
Pulmonary Langerhans' cell histiocytosis (PLCH) remains an important diagnostic consideration in the differential diagnosis of diffuse infiltrative lung disease, particular among smokers. This review highlights recent progress in our understanding of the etiology, clinical presentation, and diagnostic and therapeutic approaches to this unusual lung disorder. Evolving studies strongly link the b...
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ژورنال
عنوان ژورنال: Orphanet Journal of Rare Diseases
سال: 2014
ISSN: 1750-1172
DOI: 10.1186/s13023-014-0191-8