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AJH:脑静脉血栓形成和骨髓增生性肿瘤:74 例连续病例的三中心研究

2021-12-20 网络 网络

当前的研究使 MPN 相关的 CVT 变得清晰,并强调了它与JAK2 V617F、年龄较小和女性的密切关联。

脑静脉血栓形成 (CVT) 是一种罕见事件,一般人群中的发病率估计为每年每百万 15.7 例。脑静脉血栓形成发生在 < 1% 的 骨髓增殖性肿瘤(MPN) 患者中,但总体而言,明显的 MPN 是 3.8% 的 CVT 患者的潜在疾病,表明这两种情况之间存在联系。复发性血栓形成的预测因素和减轻与此类事件相关的过度风险的有效疗法仍有待确定。

最近脑静脉血栓形成 (CVT) 与 COVID-19 疫苗接种的关联,也促使研究者对 74 例与骨髓增殖性肿瘤 (MPN) 相关的 CVT(中位年龄 = 44 岁,范围 15-85;61% 女性)进行回顾性审查。

研究的目的:

1) 在 MPN 亚型的背景下估计 CVT 的发生率,然后描述临床表型和治疗策略;

(2) 从复发性血栓形成、主要出血事件、神经系统后遗症和生存率方面确定 MPN 合并 CVT 患者的长期前景;

(3) 确定将 MPN 相关 CVT 与 COVID 疫苗相关 CVT 区分开来的显着临床和实验室特征。

表 1: 74 名脑静脉血栓形成 (CVT) 和骨髓增生性肿瘤 (MPN) 患者的临床和实验室特征以及结果

疾病特定频率分别为 1.3% (39/2893)、1.2% (21/1811) 和 0.2% (3/1888) ,必不可少的分别为血小板增多症、真性红细胞增多症和原发性骨髓纤维化。脑静脉血栓形成发生在 ( n  = 20, 27%) 之前、 ( n  = 32, 44%) 或之后 ( n = 22) MPN 诊断。

表2: 在呈现的临床和实验室特征和42个例的结果与脑静脉血栓形成(CVT)和骨髓增殖性疾病(MPN)

 

共有 72% 的患者出现头痛。10 名 (14%) 患者的中枢神经系统出血涉及横窦 (51%)、矢状窦 (43%) 和乙状窦 (35%)。总之,91% 的受试患者携带JAK2V617F。在 19 例 (31%) 病例中确定了潜在的血栓形成倾向,在 10 例 (14%) 中确定了血栓形成史。

CVT 的治疗包括单独全身抗凝 ( n  = 27) 或联合阿司匹林 ( n  = 24)、细胞减灭疗法 ( n  = 14) 或两者 ( n  =  9))。在中位随访 5.1 年(范围 0.1-28.6)中,有 3 名(4%)患者记录到 CVT 复发,而动脉和静脉血栓复发和大出血的记录分别为 11%、9% 和 14%。后续神经学评估显示头痛 ( n  = 9)、视力丧失 ( n  = 1) 和认知障碍 ( n  = 1)。

当前的研究使 MPN 相关的 CVT 变得清晰,并强调了它与JAK2 V617F、年龄较小和女性的密切关联。区分 COVID 疫苗相关 CVT 与 MPN 相关 CVT 的临床特征包括,后者并发静脉血栓形成和脑出血的可能性较低;因此,MPN 相关的 CVT 并不致命。

 

原始出处:

Gangat, NGuglielmelli, PBetti, S, et al. Cerebral venous thrombosis and myeloproliferative neoplasms: A three-center study of 74 consecutive casesAm J Hematol20219612): 15801586. doi:10.1002/ajh.26336

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    2021-12-21 fusion
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    2022-07-29 xue8602
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