托珠单抗成功治疗误诊为IgG4相关性疾病的多中心Castleman病2例国内首报  

Two Cases of Multicentric Castleman Disease Misdiagnosed as IgG4-related Disease Were Successfully Treated with Tocilizumab: First Report in China

作　　者：陈利红[1] 施若非[1] 郑捷[1] 赵肖庆[1] 

机构地区：[1]上海交通大学医学院附属瑞金医院皮肤科,上海200025

出　　处：《中国皮肤性病学杂志》2017年第12期1285-1289,共5页The Chinese Journal of Dermatovenereology

基　　金：国家临床重点专科建设项目(2012649)

摘　　要：目的通过2例容易误诊的病例,介绍多中心Castleman病(MCD)的诊断、鉴别诊断和治疗方法。方法报道2例曾被误诊为Ig G4相关性疾病的MCD,国内首次报道使用IL-6受体抗体(托珠单抗)成功治疗该病。结果例1男,躯干泛发紫红色斑片10个月;例2女,全身紫褐色斑块17年,2例患者均伴有淋巴结肿大、贫血、发热,根据血清学检测、淋巴结活检病理和免疫组织化学、皮肤活检病理、影像学评估等确诊MCD,给予系统性糖皮质激素联合沙利度胺治疗疗效不佳,改用托珠单抗治疗,现完全缓解。结论 MCD属于罕见疾病,通过临床症状、组织病理、特殊染色及免疫组织化学等可明确诊断。对于HHV-8阴性、HIV阴性的MCD,抗IL-6治疗联合小剂量糖皮质激素可取得长期缓解。Objective By reporting 2 misdiagnosed cases,we introduced the diagnosis,differential diagnosis and treatments of multicenter Castleman disease（MCD）. Methods Two cases of MCD misdiagnosed as IgG4-related disease,successfully treated with anti-IL-6R monoclonal antibody（Tocilizumab）,were first reported in China. Results Case 1 was a male with diffuse purple and red patches on the trunk for 10 months. Case 2 was a female with purple brown plaques all over the body for 17 years. Both cases had adenopathy,anemia and fever. According to the results of blood tests,histopathology and immunohistochemistry of lymph node,skin biopsy and radiology,the diagnosis of MCD was made. They did not respond well to treatments with systemic corticosteroid combined with thalidomide. But,treatments with Tocilizumab induced complete remission in both patients. Conclusion MCD is a rare disease. Clinical manifestations,blood tests,histopathology and immunohistochemistry are helpful in the diagnosis. For the MCD with HHV-8 and HIV negative,anti-IL-6 treatment combined with low-dose glucocorticoids could result in long-term remission.

关 键 词：CASTLEMAN病 血管滤泡性淋巴结增生症 IGG4相关性疾病 托珠单抗 

分 类 号：R551.2[医药卫生—血液循环系统疾病]