Castleman病研究进展

郭美玉; 董玉君

doi:10.13201/j.issn.1004-2806.2022.07.017

Castleman病研究进展

郭美玉¹,
董玉君^1, ,

- 北京大学第一医院血液科(北京，100034)
基金项目:
北京市科委“首都临床特色应用研究”专项项目(No：Z181100001718206)

详细信息

通讯作者: 董玉君，E-mail：dongy@hsc.pku.edu.cn

中图分类号: R733

收稿日期: 2022-01-26

刊出日期: 2022-07-01

Progress in Castleman disease research

GUO Meiyu¹,
DONG Yujun^1, ,

- Department of Hematology, Peking University First Hospital, Beijing, 100034, China

More Information

Corresponding author: DONG Yujun, E-mail: dongy@hsc.pku.edu.cn

Received Date: 26 January 2022

Publish Date: 01 July 2022

摘要

摘要: Castleman病(CD)是一种罕见的淋巴增殖性疾病，临床表现为单发或多发淋巴结肿大，伴或不伴全身多器官系统受累症状。组织学上分为透明血管型、浆细胞型及混合型，临床分为单中心型(UCD)和多中心型(MCD)。MCD又分为人类疱疹病毒8型(HHV8)相关，POEMS综合征相关和特发性MCD(iMCD)。目前CD发病机制尚未清楚，已经明确的是HHV8感染与HHV8-MCD相关，人白细胞介素6在iMCD发病中起关键作用。UCD通常是局灶性病变，症状轻微，手术切除后可完全缓解; MCD表现为多发淋巴结肿大、全身炎症性症状、血细胞减少及细胞因子风暴引起的多器官功能障碍，治疗主要采用靶向治疗、化疗、免疫调节治疗等全身治疗，预后较差。目前抗逆转录病毒疗法和利妥昔单抗的使用大大改善了HIV阳性/阴性HHV8-MCD患者的预后，抗IL-6靶向治疗对许多iMCD患者有效，但对于难治性患者仍需探索新的治疗方案。近期多个组织发布了CD相关的诊断和治疗共识，对CD研究和诊疗起到重要促进作用，也越来越受到临床医生的关注。
- Castleman病 /
- 发病机制 /
- 组织病理学 /
- 诊断 /
- 治疗
Abstract: Castleman disease(CD) is a rare lymphoproliferative disorder that clinically presents with single or multiple lymph node enlargement with or without symptoms of multi-organ or system involvement. CD is histologically classified as clear vascular, plasma cell and mixed subtype, and clinically classified as unicentric(UCD) or multicentric(MCD). MCD is subdivided into human herpesvirus type 8(HHV8) -associated MCD, POEMS syndrome-associated MCD, and idiopathic MCD(iMCD). The pathogenesis of CD is unclear, but it is established that HHV8 is associated with HHV8-MCD and human interleukin 6 plays a key role in the pathogenesis of iMCD. UCD is usually a focal lesion with mild symptoms and can be completely resolved after surgical excision. MCD presents with multiple lymph node enlargements, systemic inflammatory symptoms, cytopenia and multi-organ dysfunction caused by cytokine storm. The treatment of MCD is mainly systemic therapy such as targeted therapy, chemotherapy and immunomodulatory therapy, but its prognosis is poor. The current use of antiretroviral therapy and rituximab has greatly improved the prognosis of HIV-positive/negative HHV8-MCD patients, and anti-IL-6 targeted therapy is effective for many iMCD patients, but new treatment options still need to be explored for refractory cases. Several CD-related diagnostic and therapeutic consensus have been published recently, playing an important role in promoting CD research and treatment and receiving more and more attention from clinicians.
- Castleman disease /
- pathogenesis /
- histopathology /
- diagnosis /
- treatment

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