Department of Hematology and Clinical Immunology, Yokohama City University School of Medicine, Yokohama, Japan.
Clinical Laboratory Department, Yokohama City University Hospital, Yokohama, Japan.
J Clin Exp Hematop. 2024 Sep 28;64(3):232-236. doi: 10.3960/jslrt.24006. Epub 2024 Jul 31.
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis with diverse clinical features. It is characterized by systemic histiocyte infiltration of the bone, skin, central nervous system, lung, kidney, and cardiovascular system. Pericardial involvement is frequently revealed through either pericardial effusion or pericardial thickening in patients with ECD. Although most patients remain asymptomatic, progressive pericarditis, effusion, or cardiac tamponade may occur. Herein, we report a rare and unusual presentation of ECD in a 51-year-old man who experienced severe constrictive pericarditis. The patient presented with uncontrolled fluid retention and heart failure. After the diagnosis of ECD, interferon alpha treatment was administered. The patient recovered dramatically with decreased pleural and pericardial effusion, as well as improvements in the echocardiographic signs of constrictive pericarditis. Despite several therapeutic options described in the literature for managing ECD-related pericardial disease, a standard treatment has not been established. This report highlights the importance of early treatment based on accurate diagnosis of an unusual ECD complication.
厄尔-道伊姆-切斯特病（ECD）是一种罕见的非朗格汉斯细胞组织细胞增生症，具有多种临床特征。其特征为全身组织细胞浸润骨骼、皮肤、中枢神经系统、肺、肾脏和心血管系统。心包受累通常通过 ECD 患者的心包积液或心包增厚来揭示。尽管大多数患者无症状，但可能会发生进行性心包炎、积液或心脏压塞。在此，我们报告了一例罕见的、不寻常的 51 岁男性 ECD 病例，他患有严重的缩窄性心包炎。该患者表现为无法控制的液体潴留和心力衰竭。在诊断为 ECD 后，给予干扰素 α 治疗。患者的胸腔和心包积液明显减少，缩窄性心包炎的超声心动图征象也得到改善，病情显著恢复。尽管文献中描述了多种治疗 ECD 相关心包疾病的方法，但尚未确立标准治疗方法。本报告强调了基于对不寻常 ECD 并发症的准确诊断进行早期治疗的重要性。
