The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry.
The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry.
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ACCELERATE登记研究中具有致死性结局的Castleman病患者的病程。
DOI：
10.1111/bjh.18214
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发表时间：
2022-07
影响因子：
6.5
通讯作者：
van Rhee, Frits
中科院分区：
文献类型：
--
作者：
Fajgenbaum, David C.;Pierson, Sheila K.;Kanhai, Karan;Bagg, Adam;Alapat, Daisy;Lim, Megan S.;Lechowicz, Mary Jo;Srkalovic, Gordan;Uldrick, Thomas S.;van Rhee, Frits
Castleman 病 (CD) 描述了一组罕见的、可能致命的淋巴组织增生性疾病。为了确定与 CD 死亡率相关的因素，我们分析了 ACCELERATE 登记中已故患者的数据，并将其与匹配的对照进行比较。我们分析了所有已故 CD 患者、匹配对照者和特发性多中心 Castleman 病 (iMCD) 患者亚组的人口统计、治疗和实验室数据。在 ACCELERATE 的 140 名确诊 CD 患者中，有 10 人死亡。确诊 iMCD 患者 72 例；六人死亡。与匹配对照组相比，已故 CD 队列每年住院次数更多、总体住院次数更高、每月住院天数更多，并且每年接受的治疗方案更多。实验室数据分析显示，与对照组相比，死亡队列的第 3 个月和第 6 个月的绝对淋巴细胞计数显着下降。在 iMCD 患者中，死亡组中 iMCD-TAFRO（血小板减少、全身水肿、发热、网状蛋白骨髓纤维化、肾功能不全和器官肿大）病例的比例较高。死亡 iMCD 组的免疫球蛋白 M、国际标准化比率和血小板计数显着降低。这些数据表明，致命和非致命结果的患者之间可能存在差异，并为进一步评估的参数提供了初步建议。
Castleman disease (CD) describes a group of rare, potentially fatal lymphoproliferative disorders. To determine factors associated with mortality in CD, we analysed data from deceased patients in the ACCELERATE registry and compared them with matched controls. We analysed demographic, treatment and laboratory data from all deceased CD patients, matched controls and a subgroup of idiopathic multicentric Castleman disease (iMCD) patients. Of the 140 patients in ACCELERATE with a confirmed CD diagnosis, 10 had died. There were 72 patients with confirmed iMCD; six were deceased. The deceased CD cohort had more hospitalisations per year, higher overall hospitalisations and more days hospitalised per month, and received more treatment regimens per year than the matched‐control group. Analysis of laboratory values showed a significantly decreased absolute lymphocyte count at months 3 and 6 in the deceased cohort compared with controls. Among iMCD patients, there was a higher proportion of iMCD‐TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction and organomegaly) cases in the deceased group. The deceased iMCD group had significantly lower immunoglobulin M, international normalised ratio and platelet count. These data demonstrate that there may be differences between patients who have fatal and non‐fatal outcomes, and provide preliminary suggestions for parameters to evaluate further.
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