多中心型Castleman病伴IgG4升高一例并文献复习

目的:探讨多中心型Castleman病（MCD）的诊断及治疗。方法:分析杭州市第一人民医院2020年7月收治的1例MCD患者的诊治过程，并进行相关文献复习。结果:患者，男性，55岁，贫血，球蛋白水平升高，血清IgG4>10 g/L，淋巴结肿大，既往7年未确诊。淋巴结活组织检查示，大量多克隆浆细胞增生，IgG4与IgG比值<0.40；血清白细胞介素6（IL-6）>6 000 pg/ml，诊断为MCD（浆细胞型）。RCD（利妥昔单抗+环磷酰胺+地塞米松）方案治疗效果不佳，改为抗IL-6受体的抗体托珠单抗治疗2个疗程，获得良好疗效。结论:Castleman病为一类预后较差的罕见病，异质性大，临床易误诊，确诊需依靠病理学检查。IL-6被认为与Castleman病发病密切相关，是治疗的有效靶点。

Multicentric Castleman disease with high IgG4 level: report of one case and review of literature

Objective:To investigate the diagnosis and treatment of multicentric Castleman disease (MCD).Methods:The diagnosis and treatment of one MCD patient admitted in Hangzhou First People's Hospital in July 2020 was analyzed and related literatures were reviewed.Results:The patient was a 55-year-old male with anemia, elevated globulin levels and IgG4 > 10 g/L, and enlarged lymph nodes. He was undiagnosed for 7 years. Lymph node biopsy revealed a large number of polyclonal plasma cell hyperplasia, and the ratio of IgG4/IgG was less than 0.40; the serum interleukin (IL)-6 was more than 6 000 pg/ml and then he was eventually diagnosed as MCD (plasma cell type). Rituximab + cyclophosphamide + dexamethasone (RCD) regimen was not effective, and it was changed to anti-IL-6 receptor antibody tocilizumab for 2 courses and then the patient obtained good results.Conclusions:Castleman disease is a rare disease with a poor prognosis. It has high heterogeneity and is easy to be misdiagnosed clinically. The diagnosis requires pathological examination. IL-6 is considered to be closely related to the onset of Castleman disease and has become an effective target for treatment.