Marson P, Monti G, Montani F, Riva A, Mascia M T, Castelnovo L, Filippini D, Capuzzo E, Moretto M, D'Alessandri G, Marenchino D, Zani R, Fraticelli P, Ferri C, Quartuccio L, De Silvestro G, Oreni L, Accorsi P, Galli M
Apheresis Unit, Department of Transfusion Medicine, University Hospital of Padova, Padova, Italy.
Internal Medicine, Hospital of Saronno, Saronno, Italy.
Transfus Apher Sci. 2018 Oct;57(5):639-645. doi: 10.1016/j.transci.2018.06.005. Epub 2018 Jun 26.
To assess the effectiveness of apheresis therapy (AT) in treating the clinical manifestations of patients with complicated cryoglobulinemic vasculitis (CV).
A retrospective cohort study of 159 CV patients attending 22 Italian Centers who underwent at least one AT session between 2005 and 2015. The response to AT was evaluated on the basis of a defined grading system.
Peripheral neuropathy was the most frequent clinical condition leading to AT. Therapeutic plasma exchange was used in 70.4% of cases. The outcome of AT was rated very good in 19 cases, good in 64, partial/transient in 40, and absent/not assessable in 36. Life-threatening CV-related emergencies and renal impairment independently correlated with failure to respond to AT. The independent variables associated with an increased risk of death were age at the time of the first AT session, multi-organ life-threatening CV, the presence of renal impairment and failure to respond to AT. The time-dependent probability of surviving until CV-related death in the second year was 84%, with an AHR in patients with absent/not assessable response to AT of 11.25.
In this study AT is confirmed to be a safe procedure in patients with CV. Early AT should be considered in patients with severe CV, especially in cases with impending renal involvement, in order to prevent irreversible kidney damage. Although its efficacy in patients with multi-organ failure is limited, AT is the only treatment that can rapidly remove circulating cryoglobulins, and should be considered an emergency treatment.
评估单采术（AT）治疗复杂性冷球蛋白血症性血管炎（CV）患者临床表现的有效性。
对2005年至2015年间在22个意大利中心就诊且接受至少一次AT治疗的159例CV患者进行回顾性队列研究。根据既定的分级系统评估对AT的反应。
周围神经病变是导致AT治疗的最常见临床情况。70.4%的病例使用了治疗性血浆置换。AT的结果评定为非常好的有19例，好的有64例，部分/短暂有效的有40例，无反应/不可评估的有36例。危及生命的CV相关紧急情况和肾功能损害与对AT治疗无反应独立相关。与死亡风险增加相关的独立变量为首次进行AT治疗时的年龄、多器官危及生命的CV、肾功能损害的存在以及对AT治疗无反应。第二年存活至CV相关死亡的时间依赖性概率为84%，对AT治疗无反应/不可评估的患者调整后风险比为11.25。
在本研究中，AT被证实对CV患者是一种安全的治疗方法。对于重症CV患者，尤其是有即将发生肾脏受累的情况，应考虑早期进行AT治疗，以防止不可逆转的肾脏损害。尽管AT对多器官衰竭患者的疗效有限，但它是唯一能快速清除循环冷球蛋白的治疗方法，应被视为一种紧急治疗手段。
