Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Blood Adv. 2024 Nov 12;8(21):5598-5602. doi: 10.1182/bloodadvances.2024013728.
According to the diagnostic criteria for human herpesvirus 8 (HHV-8)-negative/idiopathic multicentric Castleman disease (iMCD) proposed by Castleman Disease Collaborative Network in 2017, there is a group of HHV-8-negative patients with multicentric Castleman disease (MCD) who do not have symptoms and hyperinflammatory state and do not meet the iMCD criteria. This retrospective study enrolled 114 such patients, described as asymptomatic MCD (aMCD), from 26 Chinese centers from 2000 to 2021. With a median follow-up time of 46.5 months (range, 4-279 months), 6 patients (5.3%) transformed to iMCD. The median time between a diagnosis of aMCD and iMCD in these 6 patients was 28.5 months (range, 3-60). During follow-up, 7 patients died; 3 of them died from progression of MCD. Despite that, 37.7% of patients received systemic treatment targeting MCD; this strategy was neither associated with a lower probability of iMCD transformation nor a lower death rate. The 5-year estimated survival of all patients with aMCD was 94.1% (95% confidence interval, 88.8-99.6). Transformation to iMCD was an important predictor of death (log-rank P = .01; 5-year estimated survival, 83.3%). This study suggests that patients with aMCD may represent a potential early stage of iMCD, who may not require immediate treatment but should be closely monitored.
根据 2017 年 Castleman 疾病协作网提出的人类疱疹病毒 8 阴性/特发性多中心 Castleman 病（iMCD）的诊断标准，存在一组 HHV-8 阴性多中心 Castleman 病（MCD）患者，他们没有症状和炎症状态过高，不符合 iMCD 标准。这项回顾性研究纳入了 2000 年至 2021 年期间来自 26 个中国中心的 114 名此类患者，将其描述为无症状 MCD（aMCD）。中位随访时间为 46.5 个月（范围，4-279 个月），有 6 名患者（5.3%）转化为 iMCD。这 6 名患者从 aMCD 诊断到 iMCD 的中位时间为 28.5 个月（范围，3-60）。随访期间，有 7 名患者死亡；其中 3 人死于 MCD 进展。尽管如此，仍有 37.7%的患者接受了针对 MCD 的系统性治疗；这种策略与 iMCD 转化的可能性降低或死亡率降低均无关。所有 aMCD 患者的 5 年估计生存率为 94.1%（95%置信区间，88.8-99.6）。转化为 iMCD 是死亡的重要预测因素（对数秩 P =.01；5 年估计生存率为 83.3%）。这项研究表明，aMCD 患者可能代表 iMCD 的潜在早期阶段，他们可能不需要立即治疗，但应密切监测。
