Okamoto Shinichiro
Division of Hematology, Department of Internal Medicine, Keio University School of Medicine.
Rinsho Ketsueki. 2019;60(9):1205-1211. doi: 10.11406/rinketsu.60.1205.
Multicentric Castleman disease (MCD) is a rare polyclonal lymphoproliferative disease that causes systemic inflammation. In Western countries, human herpesvirus 8 (HHV-8) is frequently associated with MCD pathogenesis, especially in human immunodeficiency virus (HIV)-positive cases, whereas HHV-8 is seldom related to MCD cases in Japan and is referred to as idiopathic MCD (iMCD). iMCD can present as a variety of systemic symptoms and characteristic laboratory abnormalities due to IL-6 overproduction, occasionally causing organ failure. Although there have been no recent studies other than pathologic studies of iMCD, its association with TAFRO (thrombocytopenia, anasarca, reticulin fibrosis of the bone marrow, renal dysfunction, and organomegaly) syndrome has recently attracted attention in Japan as well as internationally. In 2015, a research team for iMCD published a medical reference guide and organized regional core hospitals in Japan for the diagnosis and treatment of iMCD. As a result, iMCD was designated the 331st intractable disease by the Japanese Ministry of Health, Labor and Welfare. iMCD can be expected to have a good prognosis if properly diagnosed and treated. We are optimistic that iMCD research will aid the development of medical treatment for this disease.
多中心Castleman病（MCD）是一种罕见的多克隆性淋巴增殖性疾病，可引起全身炎症。在西方国家，人类疱疹病毒8型（HHV-8）常与MCD的发病机制相关，尤其是在人类免疫缺陷病毒（HIV）阳性病例中，而在日本，HHV-8很少与MCD病例相关，被称为特发性MCD（iMCD）。由于白细胞介素-6过度产生，iMCD可表现为多种全身症状和特征性实验室异常，偶尔会导致器官衰竭。尽管除了iMCD的病理研究外，最近没有其他研究，但它与TAFRO（血小板减少、全身水肿、骨髓网状纤维增生、肾功能不全和器官肿大）综合征的关联最近在日本和国际上都引起了关注。2015年，一个iMCD研究团队发布了一份医学参考指南，并在日本组织了区域核心医院进行iMCD的诊断和治疗。结果，iMCD被日本厚生劳动省指定为第331种难治性疾病。如果得到正确的诊断和治疗，iMCD有望有良好的预后。我们乐观地认为，iMCD研究将有助于这种疾病的医学治疗发展。
