Erdheim-Chester disease with cardiac involvement: a new case report

被引:1
作者
Dogan, Riza [1 ]
Aydin, Ahmet [1 ]
Temel, Hale [1 ]
Ozer, Nejla [2 ]
Onder, Sevgen [3 ]
Hazirolan, Tuncay [4 ]
机构
[1] Hacettepe Univ, Fac Med, Dept Thorac & Cardiovasc Surg, TR-06100 Ankara, Turkey
[2] Hacettepe Univ, Fac Med, Dept Cardiol, TR-06100 Ankara, Turkey
[3] Hacettepe Univ, Fac Med, Dept Pathol, TR-06100 Ankara, Turkey
[4] Hacettepe Univ, Fac Med, Dept Radiol, TR-06100 Ankara, Turkey
来源
TURK GOGUS KALP DAMAR CERRAHISI DERGISI-TURKISH JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY | 2013年 / 21卷 / 01期
关键词
Erdheim-chester disease; non-Langerhans cell histiocytosis; osteosclerosis; pericardial effusion; MAGNETIC-RESONANCE; PATIENT;
D O I
10.5606/tgkdc.dergisi.2013.6459
中图分类号
R61 [外科手术学];
学科分类号
摘要
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis of unknown etiology, characterized by multiple organ involvement. Erdheim-Chester disease is usually diagnosed on the basis of characteristic radiologic and histopathological findings. Lesions may be skeletal or extraskeletal and may involve the skin, lung, heart, and central nervous system. In this article, we report a 62-year-old male case admitted with the complaints of cough and dyspnea. Imaging studies showed multiple osteosclerotic lesions of the bones, a large amount of pericardial effusion, and a mass which surrounds the right atrium. Cytological examination of the pericardial effusion material revealed foamy histiocytes with multinuclear giant cells. Pericardium and internal mammary artery biopsies showed fibrosis with infiltrating foamy histiocytes which were MAC38(+) and S100, CD1a(-) immunohistochemically. Based on these findings, the patient was diagnosed as ECD with extraskeletal manifestations and treated with systemic corticosteroids and chemotherapy (cyclophosphamide).
引用
收藏
页码:127 / 132
页数:6
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