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[Castleman病患者的临床特征与预后]

[Clinical Features and Prognosis of Patients with Castleman's Disease].

作者信息

Huang Xiu-Juan, Zhang Xin-Lian, Wei Xiao-Fang, Liang Xiao-Qin, Fu Yuan, Zhao Yang-Yang, Li Qing-Fen, Zhang Qi-Ke, Feng You-Fan

机构信息

Department of Hematology, Gansu Provincial Hospital; Lanzhou 730000, Gansu Province, China.

Department of Hematology, The First People's Hospital of Lanzhou City; Lanzhou 730000, Gansu Province, China.

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2023 Feb;31(1):135-140. doi: 10.19746/j.cnki.issn.1009-2137.2023.01.022.

DOI:10.19746/j.cnki.issn.1009-2137.2023.01.022
PMID:36765490
Abstract

OBJECTIVE

To analyze the clinical features and prognosis of patients with Castleman's disease (CD) and improve the diagnosis and treatment of CD.

METHODS

Clinical data of patients diagnosed with CD by pathological biopsy in Gansu Provincial Hospital from January 2009 to November 2020 were retrospectively analyzed. According to clinical classification, the patients were divided into two groups: UCD (unicentric CD) group (=20) and MCD (multicentric CD) group (=9). The clinical manifestations, laboratory examination, treatment regimens, pathological examination and follow-up data were statistically analyzed.

RESULTS

There were no significant differences in average age and gender ratio between UCD group and MCD group. In UCD patients, 80.0% were hyaline vascular type, and 20.0% were plasma cell type. In MCD patients, 33.3% were hyaline vascular type, 55.6% were plasma cell type, and 11.1% were mixed type. There was significant difference in pathological classification between the two groups (P=0.039). The UCD patients usually presented asymptomatic single lymph node enlargement with mild clinical symptoms, while the MCD patients were characterized by multiple superficial and deep lymph node enlargement throughout the body. The incidences of asthenia, splenomegaly, serous effusion in MCD group were higher than those in UCD group (<0.05). Meanwhile, the incidences of anemia, hypoproteinemia, increased ESR, elevated serum globulin and elevated β-microglobulin were significantly higher than those in UCD group too (<0.05). There was no significant difference in the incidences of abnormal WBC, PLT and elevated LDH between the two groups (>0.05). Among 20 patients with UCD, 13 cases reached complete remission (CR), 1 case achieved partial remission (PR). Among 9 patients with MCD, 3 cases received CR and 4 cases received PR.

CONCLUSION

Patients with CD requires pathological examination for diagnosis. Patients with UCD show mild clinical symptoms, good surgical treatment effect and good prognosis. Patients with MCD have diversified clinical manifestations and relatively poor prognosis, and these patients require comprehensive treatment.

摘要

目的

分析Castleman病(CD)患者的临床特征及预后,提高CD的诊断与治疗水平。

方法

回顾性分析2009年1月至2020年11月在甘肃省人民医院经病理活检确诊为CD患者的临床资料。根据临床分型,将患者分为两组:单中心型Castleman病(UCD)组(=20例)和多中心型Castleman病(MCD)组(=9例)。对临床表现、实验室检查、治疗方案、病理检查及随访资料进行统计学分析。

结果

UCD组与MCD组平均年龄及性别比差异无统计学意义。UCD患者中,透明血管型占80.0%,浆细胞型占20.0%。MCD患者中,透明血管型占33.3%,浆细胞型占55.6%,混合型占11.1%。两组病理分型差异有统计学意义(P=0.039)。UCD患者通常表现为无症状的单个淋巴结肿大,临床症状较轻,而MCD患者的特点是全身多处浅表和深部淋巴结肿大。MCD组乏力、脾肿大、浆膜腔积液的发生率高于UCD组(<0.05)。同时,MCD组贫血、低蛋白血症、血沉增快、血清球蛋白升高及β2微球蛋白升高的发生率也显著高于UCD组(<0.05)。两组白细胞、血小板异常及乳酸脱氢酶升高的发生率差异无统计学意义(>0.05)。20例UCD患者中,13例达到完全缓解(CR),1例部分缓解(PR)。9例MCD患者中,3例CR,4例PR。

结论

CD患者需经病理检查确诊。UCD患者临床症状轻,手术治疗效果好,预后良好。MCD患者临床表现多样,预后相对较差,需综合治疗。

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