title: Rapid progression to cardiac tamponade in Erdheim-Chester disease despite treatment with interferon alpha.
abstract: 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.
authors.affiliation: a Department of Internal Medicine A , Bnai Zion Medical Center, Faculty of Medicine , Technion , Haifa , Israel.
authors.forename: Afif
authors.initials: A
authors.lastname: Nakhleh
authors.affiliation: a Department of Internal Medicine A , Bnai Zion Medical Center, Faculty of Medicine , Technion , Haifa , Israel.
authors.forename: Gleb
authors.initials: G
authors.lastname: Slobodin
authors.affiliation: a Department of Internal Medicine A , Bnai Zion Medical Center, Faculty of Medicine , Technion , Haifa , Israel.
authors.forename: Nizar
authors.initials: N
authors.lastname: Elias
authors.affiliation: b Department of Pathology , Bnai Zion Medical Center, Faculty of Medicine , Technion , Haifa , Israel.
authors.forename: Jacob
authors.initials: J
authors.lastname: Bejar
authors.affiliation: a Department of Internal Medicine A , Bnai Zion Medical Center, Faculty of Medicine , Technion , Haifa , Israel.
authors.forename: Majed
authors.initials: M
authors.lastname: Odeh
mesh_terms: D000368:Aged; D002305:Cardiac Tamponade / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D004452:Echocardiography / Q000379:methods; D031249:Erdheim-Chester Disease / Q000150:complications / Q000188:drug therapy; D017809:Fatal Outcome; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage / Q000009:adverse effects; D007159:Immunologic Tests / Q000379:methods; D016898:Interferon-alpha / Q000008:administration & dosage / Q000009:adverse effects; D008297:Male; D009102:Multiple Organ Failure / Q000175:diagnosis / Q000209:etiology; D020519:Pericardiocentesis / Q000379:methods*; D010496:Pericardium / Q000473:pathology; D013902:Radiography, Thoracic / Q000379:methods; D014057:Tomography, X-Ray Computed / Q000379:methods
chemical_list: D007155:Immunologic Factors; D016898:Interferon-alpha
keywords: Erdheim–Chester disease; Interferon alpha; Pericarditis tamponade