کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2998643 1180254 2014 12 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Erdheim-Chester Disease: Characteristics and Management
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی کاردیولوژی و پزشکی قلب و عروق
پیش نمایش صفحه اول مقاله
Erdheim-Chester Disease: Characteristics and Management
چکیده انگلیسی
Erdheim-Chester disease is a rare CD68+, CD1a− non-Langerhans cell histiocytosis with multiorgan involvement. The etiology of Erdheim-Chester disease is unclear; there are no known associated infectious or hereditary genetic abnormalities. However, somatic BRAF mutations have recently been identified in these patients. Historically, the literature regarding the management of Erdheim-Chester disease consisted of case reports and small case series with anecdotal therapeutic responses to agents including, but not limited to, cytotoxic chemotherapy, bone marrow transplantation, cladribine, corticosteroids, IFN-α, the BCR-ABL/KIT inhibitor imatinib mesylate, the IL-1 receptor antagonist anakinra, the TNF-inhibitor infliximab, and recently the BRAF inhibitor vemurafenib. We performed a search of the literature using PubMed with the terms Erdheim Chester disease, without date limitations, including case reports, case series, original articles, and previous review articles. In the absence of large-scale studies, experience-based management prevails. The present review details our approach to the management of patients with Erdheim-Chester disease.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Mayo Clinic Proceedings - Volume 89, Issue 7, July 2014, Pages 985-996
نویسندگان
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