Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise.
Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise.
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DOI：
10.3390/jcm11237115
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发表时间：
2022-11-30
影响因子：
3.9
通讯作者：
中科院分区：
文献类型：
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作者：
背景：先天性血栓性血小板减少性紫癜(CTTP)是一种罕见的疾病，由ADAMTS13的遗传缺陷引起，发病率不到每百万人中有一人。在怀孕期间被诊断为TTP的患者增加了母婴并发症的风险，包括胎儿死亡。我们提出一个病例，32岁的G3P0(妊娠3，第0段)，在妊娠20周时出现先天性TTP(CTTP)和胎儿死亡的新诊断。方法：采用血小板促凝血膜动力学分析和定量蛋白质组学研究方法，对1例cTTP患者妊娠并发症的病理生理学进行描述，并与4例妊娠高血压综合征、4例先兆子痫和4例正常妊娠对照进行比较。结果：CTTP患者P-选择素、组织因子表达增加，血小板和中性粒细胞膜联蛋白-V结合增加，凝血酶生成局部性增加，提示高凝状态。在上调的15个蛋白质中，S100A8和S100A9明显过表达。结论：在我们的cTTP合并胎儿死亡的病例中，存在着血小板-中性粒细胞的激活和相互作用、血小板的高凝和前炎症。
Background: Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare disorder caused by an inherited genetic deficiency of ADAMTS13 and affects less than one per million individuals. Patients who are diagnosed with TTP during pregnancy are at increased risk of maternal and fetal complications including fetal demise. We present a case of a 32-year-old G3P0 (gravida 3, para 0) who presented at 20 weeks gestation with a new diagnosis of congenital TTP (cTTP) and fetal demise. Methods: We describe the pathophysiology of pregnancy complications in a patient with cTTP using platelet procoagulant membrane dynamics analysis and quantitative proteomic studies, compared to four pregnant patients with gestational hypertension, four pregnant patients with preeclampsia, and four healthy pregnant controls. Results: The cTTP patient had increased P-selectin, tissue factor expression, annexin-V binding on platelets and neutrophils, and localized thrombin generation, suggestive of hypercoagulability. Among 15 proteins that were upregulated, S100A8 and S100A9 were distinctly overexpressed. Conclusions: There is platelet-neutrophil activation and interaction, platelet hypercoagulability, and proinflammation in our case of cTTP with fetal demise.
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影响因子：
37.8
作者：
Agbani, Ejaife O.;van den Bosch, Marion T. J.;Poole, Alastair W.
通讯作者：
Poole, Alastair W.
影响因子：
3.5
作者：
Brown, Jenna;Potugari, Bindu;Chaturvedi, Shruti
通讯作者：
Chaturvedi, Shruti
影响因子：
20.3
作者：
Tarasco, Erika;Buetikofer, Lukas;Hovinga, Johanna A. Kremer
通讯作者：
Hovinga, Johanna A. Kremer
影响因子：
20.3
作者：
Alwan, Ferras;Vendramin, Chiara;Scully, Marie
通讯作者：
Scully, Marie
影响因子：
3.6
作者：
Lisman T
通讯作者：
Lisman T