Nakhleh Afif, Slobodin Gleb, Elias Nizar, Bejar Jacob, Odeh Majed
a Department of Internal Medicine A , Bnai Zion Medical Center, Faculty of Medicine , Technion , Haifa , Israel.
b Department of Pathology , Bnai Zion Medical Center, Faculty of Medicine , Technion , Haifa , Israel.
Mod Rheumatol. 2016 Jul;26(4):621-4. doi: 10.3109/14397595.2014.905235. Epub 2014 Apr 23.
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis with heterogeneous clinical manifestations. The most common presentation is bone pains typically involving the long bones. Approximately 75% of the patients develop extraskeletal involvement. Cardiac involvement is seen in up to 45% of the patients, and although, pericardial involvement is the most common cardiac pathology of this rare disease, cardiac tamponade due to ECD has been very rarely reported. We describe a case of a patient found to have ECD with multi-organ involvement and small pericardial effusion, which progressed to cardiac tamponade despite treatment with interferon alpha.
厄尔德海姆-切斯特病（ECD）是一种罕见的非朗格汉斯组织细胞增多症，临床表现多样。最常见的表现是骨痛，通常累及长骨。约75%的患者会出现骨骼外受累。高达45%的患者会出现心脏受累，虽然心包受累是这种罕见疾病最常见的心脏病理表现，但由ECD导致的心包填塞却鲜有报道。我们描述了一例被诊断为ECD且多器官受累并伴有少量心包积液的患者，尽管接受了干扰素α治疗，仍进展为心包填塞。
