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

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.
[Article in Chinese]

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 (n=20) and MCD (multicentric CD) group (n=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 (P<0.05). Meanwhile, the incidences of anemia, hypoproteinemia, increased ESR, elevated serum globulin and elevated β2-microglobulin were significantly higher than those in UCD group too (P<0.05). There was no significant difference in the incidences of abnormal WBC, PLT and elevated LDH between the two groups (P>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.

题目: Castleman病患者的临床特征及预后分析.

目的: 分析 Castleman病的临床特征及预后,以提高Castleman病的诊治水平.

方法: 回顾性分析甘肃省人民医院自2009年1月至2020年11月经病理活检诊断为Castleman病的29例患者的临床资料。根据临床分型分为两组:单中心型组(UCD,n=20)和多中心型组(MCD,n=9),对其临床表现、实验室检查、治疗方案、病理学检查和随访资料进行统计学分析.

结果: UCD组和MCD组平均年龄、男女比例的差异无统计学意义。UCD患者中透明血管型占比80.0%,浆细胞型占比20.0%;MCD患者中透明血管型占比33.3%;浆细胞型占比55.6%,混合型占比11.1%,两组病理分型差异有统计学意义(P=0.039)。UCD患者常表现为无症状的单个淋巴结肿大,临床症状轻微;MCD患者表现为全身多发浅表及深部淋巴结肿大。MCD组乏力、脾大、浆膜腔积液发生率均高于UCD组(P<0.05),同时,贫血、低蛋白血症、血沉升高、血清球蛋白升高、β2-微球蛋白升高发生率均明显高于UCD组(P<0.05)。而两组白细胞计数和血小板计数异常、乳酸脱氢酶升高发生率相比较,差异均没有统计学意义(P>0.05)。20例UCD患者中,13例达CR,1例PR。9例MCD患者中,3例获得CR,4例获得PR.

结论: Castleman病需病理检查确诊,UCD患者临床症状轻微,手术治疗效果良好且预后较好;MCD患者临床表现多样化,预后相对较差,需综合治疗.

Keywords: Castleman′s disease; clinical features; prognosis.

Publication types

  • English Abstract

MeSH terms

  • Anemia*
  • Castleman Disease* / diagnosis
  • Castleman Disease* / pathology
  • Castleman Disease* / therapy
  • Humans
  • Prognosis
  • Retrospective Studies
  • Splenomegaly