F.R.C.S., Department of Endocrine Surgery, King's College Hospital, King's Health Partners, Denmark Hill, London, SE5 9RS, UK.
Oncologist. 2011;16(9):1316-24. doi: 10.1634/theoncologist.2011-0075. Epub 2011 Jul 17.
Castleman's disease is a rare primary disease of the lymph nodes with limited available clinical information.
A systematic literature search identified 416 cases amenable to detailed analysis.
In HIV(-) patients, centricity, pathology type, the presence of symptoms, gender, and age all predict outcome in univariate analyses. The 3-year disease-free survival (DFS) rate for patients with unicentric hyaline vascular disease (49.5% of cases, class I) was 92.5%, versus 45.7% for those with multicentric plasma cell disease (20.2% of cases, class III) and 78.0% for those with any other combination (22.6% of cases, class II) (p < .0001). HIV(+) patients (class IV) exclusively presented with multicentric plasma cell disease and had a 3-year DFS rate of only 27.8%. Kaposi's sarcoma and lymphoma were observed in 59.3% and 9.4% of HIV(+) patients and in 2.6% and 3.6% of HIV(-) patients (p < .0001). Paraneoplastic pemphigus and the syndrome of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes were observed exclusively in HIV(-) patients at a rate of 1.3% and 1.8%, respectively.
Clinical, pathological, and viral markers allow for the classification of Castleman's disease into groups with markedly different outcomes and disease associations.
卡斯特曼病是一种罕见的淋巴结原发性疾病，临床资料有限。
系统文献检索确定了 416 例可进行详细分析的病例。
在 HIV(-)患者中，中心性、病理类型、症状存在、性别和年龄在单因素分析中均预测结局。具有单中心透明血管型疾病（49.5%病例，I 级）的患者 3 年无疾病生存率（DFS）为 92.5%，而具有多中心浆细胞病（20.2%病例，III 级）的患者为 45.7%，具有任何其他组合（22.6%病例，II 级）的患者为 78.0%（p<0.0001）。HIV(+)患者（IV 级）仅表现为多中心浆细胞病，3 年 DFS 率仅为 27.8%。卡波西肉瘤和淋巴瘤分别见于 59.3%和 9.4%的 HIV(+)患者，以及 2.6%和 3.6%的 HIV(-)患者（p<0.0001）。副肿瘤性天疱疮和多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变综合征仅见于 1.3%和 1.8%的 HIV(-)患者。
临床、病理和病毒标志物可将卡斯特曼病分为具有显著不同结局和疾病关联的组。
