Das Parthajit, Chowdhury Rajesh M, Roy Subhendu, Mishra Anil, Mandana Kayapanda, Mukherjee Sukumar
Consultant Rheumatologist, Apollo Multispeciality Hospital, Kolkata, West Bengal, India, Corresponding Author.
Consultant Ophthalmologist, Fortis Hospital, Kolkata, West Bengal, India.
J Assoc Physicians India. 2025 Jan;73(1):81-82. doi: 10.59556/japi.73.0778.
Rosai-Dorfman disease (RDD) is an extremely rare, histiocytic proliferative disorder most commonly presenting as lymphadenopathy in young adults and children. Although it may present with a wide range of extranodal manifestations, involvement of the cardiovascular system is extremely rare. The etiopathogenesis of RDD is poorly understood. Sporadic RDD is mostly a self-limited disease and has a good clinical outcome. There is a lack of standardized, evidence-based recommendations to treat multifocal extranodal disease. Poor prognostic factors include autoimmune cytopenias as well as disseminated organ involvement, including kidneys, heart, lower respiratory tract, liver, etc. We report a case of a 54-year-old gentleman who presented with refractory pericardial effusion, orbital disease, and lymphadenopathy, with a good clinical response to immunosuppressive therapy.
罗萨伊-多夫曼病（RDD）是一种极为罕见的组织细胞增生性疾病，最常见于青年人和儿童，表现为淋巴结病。尽管它可能伴有多种结外表现，但累及心血管系统极为罕见。RDD的发病机制尚不清楚。散发性RDD大多是一种自限性疾病，临床预后良好。目前缺乏针对多灶性结外疾病的标准化、循证治疗建议。不良预后因素包括自身免疫性血细胞减少以及累及肾脏、心脏、下呼吸道、肝脏等多个器官。我们报告一例54岁男性患者，该患者表现为难治性心包积液、眼眶疾病和淋巴结病，免疫抑制治疗后临床反应良好。
