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首页> 外文期刊>Platelets >Use of a thrombopoietin receptor agonist in von Willebrand disease type 2B (p.V1316M) with severe thrombocytopenia and intracranial hemorrhage
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Use of a thrombopoietin receptor agonist in von Willebrand disease type 2B (p.V1316M) with severe thrombocytopenia and intracranial hemorrhage

机译：血小板生成素受体激动剂在von willebrand型2b（p.v1316m）中的使用严重血小板减少症和颅内出血

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We present here a 63-year old woman with a long history of immune thrombocytopenia. She was hospitalized for a traumatic intracranial hemorrhage with thrombocytopenia. Following inefficient treatment of four platelet transfusions, immunoglobulins, and corticosteroids, we initiated treatment with a thrombopoietin (TPO) receptor agonist (eltrombopag 25 mg/d) with a good efficacy. Her mother and sister also had chronic thrombocytopenia. Clinical history, hemostasis results, and gene analysis revealed von Willebrand disease (VWD) type 2B with the mutation (c.3946G> A; p.V1316M), which combines a von Willebrand factor defect with severe thrombocytopenia, as well as a thrombocytopathy. The efficacy of TPO receptor agonists appears to counterbalance, at least to some extent, the thrombocytopathy associated with this mutation. As such, the use of TPO receptor agonists could represent an alternative therapeutic approach in cases of VWD type 2B with severe thrombocytopenia.
机译：我们在此报告一位63岁女性，长期患有免疫性血小板减少症。她因创伤性颅内出血伴血小板减少症住院。在四次血小板输注、免疫球蛋白和皮质类固醇治疗无效后，我们开始使用血小板生成素（TPO）受体激动剂（25 mg/d）进行治疗，疗效良好。她的母亲和妹妹也患有慢性血小板减少症。临床病史、止血结果和基因分析显示，血管性血友病（VWD）2B型具有突变（c.3946G>A；p.V1316M），该突变结合了血管性血友病因子缺陷、严重血小板减少症和血小板减少症。TPO受体激动剂的疗效似乎至少在一定程度上抵消了与该突变相关的血小板病变。因此，TPO受体激动剂的使用可能是治疗伴有严重血小板减少的VWD 2B型患者的一种替代方法。

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