多中心型Castleman病伴IgG4升高一例并文献复习

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

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)>6000 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。

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 6000 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.