Abstract：
Type I cryoglobulinemia (CG) accounts for 10%-15% of all cryoglobulinemias and are exclusively seen in clonal proliferative hematologic conditions. In this multicenter nationwide cohort study, we analyzed the prognosis and long-term outcomes of 168 patients with type I CG (93 (55.4%) IgM and 75 [44.6%] IgG). Five- and 10-year event-free survivals (EFS) were 26.5% (95% CI 18.2%-38.4%) and 20.8% (95% CI 13.1%-33.1%), respectively. In multivariable analysis, factors associated with poorer EFS were renal involvement (HR: 2.42, 95% CI 1.41-4.17, p = .001) and IgG type I CG (HR: 1.96, 95% CI 1.13-3.33, p = 0.016), regardless of underlying hematological disorders. IgG type I CG patients had higher cumulative incidence of relapse (94.6% [95% CI 57.8%-99.4%] vs. 56.6% [95% CI 36.6%-72.4%], p = .0002) and death at 10 years (35.8% [19.8%-64.6%] vs. 71.3% [54.0%-94.2%], p = .01) as compared to IgM CG, respectively. Overall, complete response of type I CG at 6 months was 38.7%, with no significant difference between Igs isotypes. In conclusion, renal involvement and IgG CG were identified as independent poor prognostic factors of type I CG.
摘要：
I型冷球蛋白血症（CG）占所有冷球蛋白血症的10％-15％，仅在克隆增生性血液学疾病中可见。在这项全国多中心队列研究中，我们分析了168例I型CG(93例(55.4%)IgM和75例[44.6%]IgG)患者的预后和长期结局.五年和十年无事件生存率（EFS）分别为26.5％（95％CI18.2％-38.4％）和20.8％（95％CI13.1％-33.1％），分别。在多变量分析中，与较差的EFS相关的因素是肾脏受累（HR：2.42，95％CI1.41-4.17，p=0.001）和IgGI型CG（HR：1.96，95％CI1.13-3.33，p=0.016），不管潜在的血液系统疾病。IgGI型CG患者的累积复发率较高（94.6％[95％CI57.8％-99.4％]与56.6%[95%CI36.6%-72.4%]，p=.0002）和10年死亡（35.8%[19.8%-64.6%]与71.3%[54.0%-94.2%]，p=0.01)与IgMCG相比，分别。总的来说,6个月时I型CG的完全缓解率为38.7%，Igs同种型之间无显著差异。总之,肾脏受累和IgGCG被确定为I型CG的独立不良预后因素.
