Castleman病
Castleman disease
定义 英文原文（暂无中文）
A rare lymphoid hemopathy characterized by involvement of lymph nodes in any part of the body, most frequently the mediastinum, abdomen, neck, or spleen, and occurring as unicentric, idiopathic multicentric, or KSHV/HHV8-associated multicentric Castleman disease. Depending on the type, patients are most commonly asymptomatic or typically present with systemic symptoms.
别名
血管滤泡性淋巴增生
基本事实
- 遗传方式
- 不适用
- 发病年龄
- 各年龄段
- 患病率
- 1-9 / 100 000（United States）
临床表型 29
极常见 99–80%1
- 淋巴结肿大 HP:0002716
常见 79–30%10
- 腹痛 HP:0002027
- 贫血 HP:0001903
- 全身性症状 HP:0025142
- C-反应蛋白水平升高 HP:0011227
- 红细胞沉降速度升高 HP:0003565
- 疲乏 HP:0012378
- 淋巴滤泡增生 HP:0002729
- 循环白细胞介素6水平升高 HP:0030783
- 纵膈淋巴结病 HP:0100721
- 体重减轻 HP:0001824
偶见 29–5%9
- 腹胀 HP:0003270
- 腹部肿块 HP:0031500
- 胃肠道异常 HP:0011024
- 咳嗽 HP:0012735
- 红细胞平均体积减低 HP:0025066
- 侧腹疼痛 HP:0030157
- 全身性淋巴结肿大 HP:0008940
- 黄疸 HP:0000952
- 恶心和呕吐 HP:0002017
罕见 <4–1%9
- 全身水肿 HP:0012050
- 呼吸困难 HP:0002094
- 血尿 HP:0000790
- 肠梗阻 HP:0005214
- 骨髓纤维化 HP:0011974
- 肾功能不全 HP:0000083
- 限制型心肌病 HP:0001723
- 血小板减少症 HP:0001873
- 输尿管梗阻 HP:0006000
近两年的全球研究 877L2
2024/08 起在 Europe PMC 检索所得，按发表时间倒序显示最近 20 篇。标题未译成中文——自动翻译需要接入 LLM 服务，尚未引入。
- 2026-09综述HHV-8 Infection in Solid-Organ Transplant-Pathogenesis, Epidemiology, Clinical Manifestations and Management
- 2026-08综述Evolving understanding of Kaposi sarcoma and associated diseases in the ART era
- 2026-08病例报告Diagnostic challenge of human herpesvirus 8-associated multicentric Castleman disease in a patient living with HIV disease
- 2026-08Clinicopathological features and outcomes of glomerular microangiopathy: a retrospective cohort study
- 2026-07Recognising idiopathic plasmacytic lymphadenopathy within the evolving spectrum of idiopathic multicentric Castleman disease
- 2026-07病例报告HIV-negative KSHV/HHV8-associated multicentric Castleman disease with concurrent Kaposi sarcoma and POEMS-like manifestations
- 2026-0718F-FDG and 68Ga-Pentixafor PET/CT in a Patient With Castleman Disease
- 2026-07Paraneoplastic Pemphigus/Paraneoplastic Autoimmune Multiorgan Syndrome Associated With Castleman Disease: Multidisciplinary Management of a Rare Life-Threatening Syndrome
- 2026-07POEMS Syndrome: 2026 Update on Diagnosis, Risk-Stratification, and Management
- 2026-07病例报告[EXTRAMEDULLARY HEMATOPOIESIS PRESENTING AS A RENAL MASS DURING TREATMENT OF CASTLEMAN DISEASE : A CASE REPORT]
- 2026-07病例报告[Clinical diagnosis and treatment of four cases of Castleman disease in children]
- 2026-07病例报告Sustained Complete Response in Refractory Idiopathic Multicentric Castleman Disease Following a Single CD19 CAR T-Cell Infusion: A Case Report With Mechanistic Insight
- 2026-07Stromal-immune crosstalk underlying the pathogenesis of paraneoplastic pemphigus associated with Castleman disease
- 2026-07Transcriptome profiling reveals that cellular composition contributes to the hyaline-vascular variant in unicentric Castleman disease-associated paraneoplastic pemphigus
- 2026-07Nocardia farcinica as a Potential Pathogen Associated With a Clinical Subtype of Kikuchi-Fujimoto Disease
- 2026-06病例报告POEMS syndrome: A case report
- 2026-06A Rare Case of POEMS-Associated Multicentric Castleman Disease with an Atypical Osteolytic Presentation
- 2026-06开放获取IgG4-related inflammatory pseudotumor presenting with refractory seizures: a diagnostic pitfall
- 2026-06病例报告Human Herpes Virus 8 (HHV-8)-Associated Multicentric Castleman Disease in an HIV Patient With Severe Immunosuppression: A Diagnostic Challenge
- 2026-06综述病例报告开放获取Successful treatment of idiopathic multicentric Castleman disease with TAFRO and kidney involvement: case report and literature review
国家医保药品目录中点名本病的药品 1L2
出自《国家基本医疗保险、生育保险和工伤保险药品目录（2025年）》（医保发〔2025〕33号，2026-01-01 起执行）。下列药品在药品名称或限定支付范围里出现了本病的名称。
匹配不到 ≠ 不能报销。目录里只有约一成药品设了限定支付范围，其余按适应症正常使用同样可报销；本区块只能回答「目录有没有点名这个病」，不能回答「这个病有没有药能报销」。各省执行细则、双通道与单独支付范围另有规定，请以当地医保部门口径为准。
- 注射用司妥昔单抗乙类谈判药品限人体免疫缺陷病毒(HIV)阴性和人疱疹病毒8型(HHV-8)阴性的多中心Castleman病(MCD)成人患者。
在中国开展的临床试验 8L2
按病名在 ClinicalTrials.gov 检索、并校验研究中心含中国大陆而来。登记状态不等于现在真的能入组——务必按 NCT 号到原站核实，并与主治医生商量。
登记为可入组 2
- 招募中NCT05200715AutoInflammatory Disease Alliance Registry (AIDA)中国研究中心 2 个：Beijing、Chongqing
- 招募中NCT05283993A Cohort Study of Plasma Cell Disorders (PCDs) in PKUFH中国研究中心 1 个：Beijing
其他状态的试验（6 项）
- 已完成NCT01024036A Study to Evaluate the Efficacy and Safety of CNTO328 Plus Best Supportive Care in Multicentric Castleman's Disease中国研究中心 5 个：Beijing、Chengdu、Guangzhou、Hangzhou、Shanghai
- 已完成NCT01400503A Study to Evaluate the Safety of Long-term Treatment With Siltuximab in Patients With Multicentric Castleman's Disease中国研究中心 2 个：Beijing、Chengdu
- 状态未知NCT03043105TCP Regimen in Newly Diagnosed MCD:a Prospective, Single-center, Single-arm, Phase-II Pilot Trial中国研究中心 1 个：Beijing
- 状态未知NCT03982771BCD Regimen in Newly Diagnosed Idiopathic Multicentric Castleman's Disease (iMCD)中国研究中心 1 个：Beijing
- 状态未知NCT04743687Zanuburutinib in Relapsed and Refractory iMCD: a Prospective, Single-center, Single-arm Trial中国研究中心 1 个：Beijing
- 进行中·不再招募NCT05345522A Study of Anti-IL-6R mAb Injection in Patients With iMCD中国研究中心 3 个：Beijing、Chengdu
中国境外的在招试验 13L2
这些试验在中国没有研究中心，通常无法直接报名——入组一般要求在当地居住并接受随访。列在这里是因为它另有用处：看清楚全世界正在试哪些药、做到了哪一期、由谁在做。把药名和 NCT 编号记下来去问主治医生，或据此进一步查该药是否已在境外获批、是否有拓展性用药（expanded access）通道。
美国9意大利1
CT.gov 报告命中 13 项，此处取回并展示最近的 11 项。
- 招募中NCT07085039Ruxolitinib in Previously Treated Idiopathic Multicentric Castleman Disease美国
- 招募中NCT06052618Phase II Study of Pacritinib in Kaposi Sarcoma Herpesvirus (KSHV)-Associated Multicentric Castleman Disease and KSHV-Associated Inflammatory Cytokine Syndrome (KICS)美国
- 尚未开始招募NCT06643091Nintedanib Treatment in Unicentric Castleman Disease
- 招募中NCT05907759Daratumumab for Relapsed/Refractory Primary Effusion Lymphoma, Plasmablastic Lymphoma, and Multicentric Castleman Disease美国
- 招募中NCT05663502Collecting Blood and Tissue Sample Donations for Research for HIV/AIDS-Related Cancers美国
- 招募中NCT06720870An Italian Multicenter Retrospective Observational Study to Assess Effectiveness and Safety of Siltuximab for Patients with Castleman's Disease Treated in Italy in a Real-life Context意大利
- 招募中NCT04968288Natural History of KSHV-Associated Multicentric Castleman s Disease美国
- 招募中NCT03717844Registry for Adults With Plasma Cell Disorders (PCD's)美国
- 招募中NCT03300830Molecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease美国
- 招募中NCT02817997International Registry for Patients With Castleman Disease美国
- 招募中NCT00006518Specimen Collections From Participants With HIV Infection, KSHV Infection, Viral-Related Pre-malignant Lesions and Cancer美国
外部标识与链接
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本页数据来源
- 疾病定义、同义词、基因、表型、流行病学：Orphanet（CC BY 4.0）
- 中文病名：Orphanet 中文包，冻结于 2020-06-01
- 表型中文标签：HPO 简体中文翻译（CHPO 上游成果）