Tang Dijiao, Guo Yuetong, Tang Yi, Wang Hongxu
Department of Laboratory Medicine, The First Affiliated Hospital of Chongqing Medical University, Chongqing, People's Republic of China.
Department of Pathology, Chongqing Medical University, Chongqing, People's Republic of China.
Ther Clin Risk Manag. 2022 Apr 26;18:499-509. doi: 10.2147/TCRM.S354130. eCollection 2022.
Castleman disease (CD) is a rare and heterogeneous lymphoproliferative disorder with a spectrum of characteristic pathological abnormalities of lymph node. Furthermore, its clinical diagnosis is very challenging until pathological results are available. This study aimed to investigate the clinical presentations, treatment and prognosis of CD, thereby improving the understanding and diagnosis of CD.
This study retrospectively analyzed the clinical data of 31 patients with CD admitted to the First Hospital Affiliated Hospital of Chongqing Medical University January 2013 to December 2020. The chi-square test and the Mann-Whitney rank sum test were employed to calculate between-group differences for categorical and quantitative data, respectively.
Clinically, patients with unicentric CD (UCD) usually present with lymphadenopathy. However, the clinical presentation of patients with multicentric CD (MCD) ranged from mild lymphadenopathy with B-symptoms (5/8, 62.5%) to intense inflammation, vascular leak syndrome (3/8, 37.5%), hepatosplenomegaly (3/8, 37.5%), organ insufficiency (3/8, 37.5%), and even death (2/8, 25.0%). Compared with UCD patients, patients with MCD had significantly lower levels of hemoglobin (104 (90,129) vs 137 (120,149), p=0.018) and plasma albumin (31.5 (27.0,37.0) vs 45.0 (40.0,46.5), p=0.001), but IgG levels were significantly increased. Patients with UCD were mainly treated with surgical resection alone, with a five-year survival rate of 95.65%. When siltuximab is not an option, steroid plus rituximab-based chemotherapy and specific supportive care are common options for MCD. Except for 2 deaths, the remaining MCD patients have stable disease or partial remission.
CD describes a heterogeneous group of disorders characterized by morphologically benign lymphoid hyperplasia. Notably, patients with MCD present varying degrees of inflammation responses, even involving multiple systems. Surgery is a direct and effective way to diagnose and treat UCD. In the absence of IL-6 antagonists, anti-inflammatory and immunosuppressive therapeutic strategies, and cytotoxic clearance of cells responsible for hypercytokinemia could be adopted.
卡斯特曼病（CD）是一种罕见的异质性淋巴增殖性疾病，具有一系列淋巴结特征性病理异常。此外，在获得病理结果之前，其临床诊断极具挑战性。本研究旨在探讨CD的临床表现、治疗及预后，从而提高对CD的认识和诊断水平。
本研究回顾性分析了2013年1月至2020年12月在重庆医科大学附属第一医院收治的31例CD患者的临床资料。分别采用卡方检验和曼-惠特尼秩和检验计算分类数据和定量数据的组间差异。
临床上，单中心型CD（UCD）患者通常表现为淋巴结病。然而，多中心型CD（MCD）患者的临床表现范围从伴有B症状的轻度淋巴结病（5/8，62.5%）到严重炎症、血管渗漏综合征（3/8，37.5%）、肝脾肿大（3/8，37.5%）、器官功能不全（3/8，37.5%），甚至死亡（2/8，25.0%）。与UCD患者相比，MCD患者的血红蛋白水平（104（90，129）vs 137（120，149），p = 0.018）和血浆白蛋白水平（31.5（27.0，37.0）vs 45.0（40.0，46.5），p = 0.001）显著降低，但IgG水平显著升高。UCD患者主要单独接受手术切除治疗，五年生存率为95.65%。当无法使用司妥昔单抗时，基于类固醇加利妥昔单抗的化疗及特定的支持治疗是MCD的常见治疗选择。除2例死亡外，其余MCD患者病情稳定或部分缓解。
CD描述了一组以形态学上良性淋巴样增生为特征的异质性疾病。值得注意的是，MCD患者表现出不同程度的炎症反应，甚至累及多个系统。手术是诊断和治疗UCD的直接有效方法。在缺乏IL-6拮抗剂的情况下，可采用抗炎和免疫抑制治疗策略以及对导致高细胞因子血症的细胞进行细胞毒性清除。
