Castleman's Disease: Systematic Analysis of 416 Patients from the Literature

Learning Objectives After completing this course, the reader will be able to: Describe the centricity and histopathology profiles of each of the three classes of HIV‐negative patients identified in this analysis. Correlate nosological classification and outcomes in patients with Castleman's disease. This article is available for continuing medical education credit at CME.TheOncologist.com Background. Castleman's disease is a rare primary disease of the lymph nodes with limited available clinical information. Methods. A systematic literature search identified 416 cases amenable to detailed analysis. Results. 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. Conclusion. Clinical, pathological, and viral markers allow for the classification of Castleman's disease into groups with markedly different outcomes and disease associations. 摘要 背景. Castleman 病是一种罕见的淋巴结原发疾病，其临床资料有限。 方法. 通过系统的文献检索得到416例病例，进行详细分析。 结果. 单因素分析显示，中心性[u1]、病理类型、症状、性别和年龄均可预测HIV－患者的预后。单中心透明血管型患者（占所有病例的49.5%，I型）的3年无事件生存（DFS）率为92.5%，而多中心浆细胞型患者（占所有病例的20.2%，III型）的3年DFS率为45.7%，混合型患者（占所有病例的22.6%，II型）的3年DFS率为78.0%（p