Adult Langerhans cell histiocytosis and immunomodulatory drugs:Review and analysis of thirty-four case reports

• 作者: Endri Mauro Filippo Gherlinzoni Piero Maria Stefani
• 来源期刊: 世界血液学杂志
• 学科: 医学
• 关键词: LANGERHANS cell HISTIOCYTOSIS IMMUNOMODULATORY DRUGS THALIDOMIDE LENALIDOMIDE
• 年，卷（期）: 2019,（1）
• 页码范围: 1-9
• 页数: 9页
• 分类号: R55

摘要

Langerhans cell histiocytosis(LCH)is a rare neoplastic disease in dendritic cells.LCH is classified as either a single-system(SS)or multisystem(MS)disease.There is not a standard first-line treatment for LCH in adults.We analyzed the efficacy and safety of immunomodulatory drugs(IMiDs)by searching PubMed/MEDLINE for case reports previously published.The clinical response(nonactive disease or active disease that regressed)was 94%in SS and 53%in MS.IMiDs should only be considered for adults with cutaneous SS involvement;in MS,they should be used only for patients not eligible for more aggressive treatments.