Effect of cladribine therapy on lung cysts in pulmonary Langerhans cell histiocytosis
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چکیده
Cladribine therapy may be beneficial in advanced forms of pulmonary Langerhans cell histiocytosis, even that with multiple cystic changes http://ow.ly/yeLr30i0Tt6.
منابع مشابه
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...
متن کاملEffectiveness 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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Pulmonary Langerhans’ cell histiocytosis (LCH)/histiocytosis X is a rare smoking-related interstitial lung disease that predominantly affects adults aged 20–40 yrs. It is commonly associated with irreversible airflow obstruction and highly variable clinical outcomes. The disease may regress spontaneously or following smoking cessation; however, in 20–30% of cases it progresses to chronic respir...
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Introduction & Objective: Pulmonary Langerhans Cell Histiocytosis (PLCH) is an uncommon (5% ILD) interstitial tissue disorder with significant importance. It occurs predominantly in adult smokers. The organs involved in LCH include skin, bone, pituitary gland, thyroid, lymph node, and lungs. Materials & Methods: In this descriptive study all patients admitted to the Masih Daneshvari Hospit...
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A 32-year-old man was admitted in the Emergency Department with acute dyspnea secondary to spontaneous pneumothorax. He had history of spontaneous pneumothorax 2 years ago.He was active cigarette smoker (10 pack/year). The family history was unremarkable for lung diseases. Additionally, he had no systemic complaints.The lung HRCT revealed multiple bizarre-shaped cysts distributed in both lungs ...
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