不同亚型Castleman病患者的临床特征比较

Comparison of clinical features of patients with Castleman’s disease of different subtypes

目的比较不同亚型Castleman病(CD)的临床特征。方法回顾性分析2012年1月1日至2018年7月1日郑州大学第一附属医院收治的86例CD患者的临床资料,按肿大淋巴结分布和器官受累的情况分为单中心型(UCD)和多中心型(MCD),分别为63、23例。比较UCD组和MCD组患者的确诊年龄、性别、诊断方式、病理类型、临床表现、实验室检查等指标。结果两组患者性别、年龄比较,差异无统计学意义(P>0.05)。MCD组通过手术切除进行诊断的患者占比低于UCD组,差异有统计学意义(P<0.05)。MCD组透明血管型比例低于UCD组,MCD组浆细胞型比例高于UCD组,差异有统计学意义(P<0.05)。MCD组发热、乏力、水肿、血红蛋白下降、血小板异常、低白蛋白血症的患者占比均高于UCD组,差异有统计学意义(均P<0.05)。MCD组患者白细胞异常、血沉升高、C反应蛋白升高的发生率分别与UCD组比较,差异无统计学意义(P>0.05)。UCD组和MCD组接受治疗的分别为62、18例,UCD组和MCD组药物化疗率分别为1.6%(1/62)、88.9%(16/18)。MCD组药物化疗率高于UCD组,差异有统计学意义(P<0.05)。随访完成患者包括UCD组30例和MCD组14例。UCD组死亡率和平均随访时间分别为3.3%(1/30)、(34.7±13.5)个月,MCD组死亡率和平均随访时间分别为21.4%(3/14)、(32.2±12.6)个月。两组患者死亡率、平均随访时间比较,差异无统计学意义(均P>0.05)。结论与UCD相比,MCD临床症状较重,浆细胞型比例较高,实验室检查多有异常。UCD和MCD的常用治疗方式分别为手术切除、药物化疗。

Objective To compare the clinical features of patients with Castleman’s disease of different subtypes(CD).Methods The clinical data of 86 patients with CD admitted to the First Affiliated Hospital of Zhengzhou University from January 1,2012 to July 1,2018 were retrospectively analyzed.According to the distribution of enlarged lymph nodes and organ involvement,the patients were divided into unicentric Castleman’s disease(UCD)and multicentric Castleman’s disease(MCD),with 63 and 23 cases respectively.The diagnosis age,sex,diagnosis method,pathological type,clinical manifestation,laboratory examination and other indexes of UCD group and MCD group were compared.Results There was no significant difference in gender and age between the two groups(P>0.05).The proportion of patients diagnosed by surgical resection in MCD group was lower than that in UCD group,and the difference was statistically significant(P<0.05).The proportion of hyaline-vascular type in MCD group was lower than that in UCD group,and the proportion of plasma cell type in MCD group was higher than that in UCD group,while the differences were statistically significant(P<0.05).The proportions of patients with fever,fatigue,edema,hemoglobin decline,platelet abnormality and hypoalbuminemia in MCD group were higher than that in UCD group,and the differences were statistically significant(all P<0.05).The incidences of leucocyte abnormality,increased erythrocyte sedimentation rate and elevated C-reactive protein in MCD group were not statistically significant compared with those in UCD group(P>0.05).There were 62 and 18 patients in UCD group and MCD group respectively who received treatment,and the rates of drug chemotherapy in UCD group and MCD group were 1.6%(1/62)and 88.9%(16/18)respectively.The rate of drug chemotherapy in MCD group was higher than that in UCD group,and the difference was statistically significant(P<0.05).The follow-up included 33 patients in UCD group and 20 patients in MCD group.The mortality rate and average follow-up time in UCD group were 3.3%(1/30)and(34.7±13.5)months respectively,while the mortality rate and average follow-up time in MCD group were 21.4%(3/14)and(32.2±12.6)months respectively.There were no significant differences in mortality rate and average follow-up time between the two groups(P>0.05).Conclusion Compared with UCD,MCD has more severe clinical symptoms,higher proportion of plasma cell type,and more abnormalities in laboratory examination.The common treatment methods for UCD and MCD are surgical resection and drug chemotherapy respectively.