背景:Rosai-Dorfman病是一种罕见的疾病,通常表现为结节性疾病。心脏受累非常罕见,发生在0.1-0.2%的病例中,这阻碍了我们的理解。我们报告了一例没有淋巴结受累的患者与Rosai-Dorfman病(RDD)相关的心脏表现。Further,我们对文献进行了全面回顾,以巩固有关RDD心脏表现患者通常如何管理和治疗的数据.
方法:对PubMed,WebofScience,并进行Embase以确定心脏受累的RDD病例。在确定的464项研究中,包括43例患者的42篇出版物符合标准,并纳入本综述。我们收集了病人的人口统计数据,以及他们的管理和治疗方法。此外,我们与一位出现与RDD相关的心脏质量的患者分享我们自己的经验.
结果:在43名患者中,只有20.9%(n=9)在心脏表现之前有RDD病史.据报道,淋巴结受累率为32.6%(n=14),而结外心外受累占46.5%(n=20)。在介绍时,最常见的症状是呼吸困难(48.8%,n=21),胸部不适(41.9%,n=18),和下肢水肿(16.3%,n=7)。心脏表现最常见于右心房(41.9%,n=18)和心包(18.6%,n=8)。治疗包括全身药物治疗(34.9%,n=15)和心脏手术(39.5%,n=17)。中位随访时间为12个月(1至36个月),8例患者(18.6%)死亡。我们的病人,左心房有心脏肿块的人,接受了手术切除,过去5年无任何症状复发.
结论:心脏相关的RDD表现的频率可能大于最初的感觉。这些结果强调了识别RDD及其心脏相关表现的重要性,促进受影响个体的及时诊断和治疗。
BACKGROUND: Rosai-Dorfman disease is a rare condition that typically presents as a nodal disease. Cardiac involvement is extremely uncommon, occurring in 0.1-0.2% of cases, which has hindered our understanding. We report a case of Rosai-Dorfman disease (RDD) related cardiac manifestation in a patient without nodal involvement. Further, we conduct a comprehensive
review of the literature to consolidate data on how patients with cardiac manifestations of RDD are typically managed and treated.
METHODS: A systematic
review of PubMed, Web of Science, and Embase was conducted to identify cases of RDD with cardiac involvement. Out of 464 studies identified, 42 publications encompassing 43 patients met the criteria and were incorporated in this
review. We gathered data on patient demographics, as well as their management and treatment approaches. Additionally, we share our own experience with a patient who presented with a cardiac mass related to RDD.
RESULTS: Out of the 43 patients, only 20.9% (n = 9) had a documented history of RDD prior to cardiac manifestations. Nodal involvement was reported in 32.6% (n = 14), while extranodal extracardiac involvement was reported in 46.5% (n = 20). Upon presentation, the most prevalent symptoms were dyspnea (48.8%, n = 21), chest discomfort (41.9%, n = 18), and lower extremity edema (16.3%, n = 7). Cardiac manifestations were most frequently found in the right atrium (41.9%, n = 18) and
pericardium (18.6%, n = 8). Treatment encompassed systemic medical therapy (34.9%, n = 15) and cardiac surgery (39.5%, n = 17). The median follow-up period was 12 months (with a range of 1 to 36), and 8 patients (18.6%) experienced mortality. Our patient, who had a cardiac mass in the left atrium, underwent resection and has remained symptom-free without any recurrence for the past 5 years.
CONCLUSIONS: The frequency of cardiac related-RDD manifestations may be greater than initially perceived. These results underscore the significance of identifying RDD and its cardiac-related presentations, facilitating timely diagnosis and treatment for affected individuals.