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<p>Pulmonary Langerhans cell histiocytosis is a&#xa0;rare disease primarily affecting young adults who smoke. Histologically, in addition to necrotizing granulomas with Langerhans cells there is also an increase in eosinophilic granulocytes. Radiologically, nodular changes dominate in the early stage, which develop into lung cysts of varying sizes as the disease progresses. In the late stage this can result in fibrotic remodeling and an emphysema pattern of the lung tissue due to destruction. The Langerhans cells are positive for CD1a, S100 and langerin (CD207). Electron microscopy reveals the presence of so-called Birbeck granules, which are characteristic of the disease. A&#xa0;surgical biopsy with histological confirmation is the gold standard in the diagnostics. An early diagnosis is of great importance as smoking cessation is crucial and represents an adequate treatment for many patients. In advanced and symptomatic cases, systemic treatment can be necessary, with corticosteroids being an important treatment option, especially during the inflammatory activity phase. In the presence of specific mutations (e.g., BRAF mutations), the use of targeted treatment (e.g., BRAF inhibitors) can be considered.</p>

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Langerhans-Zell-Histiozytose und andere tumoröse Auslöser von Lungenzysten

  • 期刊论文

  • 发表日期：

    2025年3月18日

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  • Elvin Atug

摘要

Pulmonary Langerhans cell histiocytosis is a rare disease primarily affecting young adults who smoke. Histologically, in addition to necrotizing granulomas with Langerhans cells there is also an increase in eosinophilic granulocytes. Radiologically, nodular changes dominate in the early stage, which develop into lung cysts of varying sizes as the disease progresses. In the late stage this can result in fibrotic remodeling and an emphysema pattern of the lung tissue due to destruction. The Langerhans cells are positive for CD1a, S100 and langerin (CD207). Electron microscopy reveals the presence of so-called Birbeck granules, which are characteristic of the disease. A surgical biopsy with histological confirmation is the gold standard in the diagnostics. An early diagnosis is of great importance as smoking cessation is crucial and represents an adequate treatment for many patients. In advanced and symptomatic cases, systemic treatment can be necessary, with corticosteroids being an important treatment option, especially during the inflammatory activity phase. In the presence of specific mutations (e.g., BRAF mutations), the use of targeted treatment (e.g., BRAF inhibitors) can be considered.