Thrombin generation abnormalities in commonly encountered platelet function disorders.
Thrombin generation abnormalities in commonly encountered platelet function disorders.
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DOI：
10.1111/ijlh.13638
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发表时间：
2021-12
影响因子：
3
通讯作者：
Hayward CPM
中科院分区：
文献类型：
--
作者：
Sharma T;Brunet JG;Tasneem S;Smith SA;Morrissey JH;Hayward CPM
利用富血小板血浆 (PRP) 和贫血小板血浆 (PPP) 进行凝血酶生成 (TG) 的研究为出血性疾病提供了见解。我们研究了一组常见血小板功能障碍 (PFD) 患者的 TG。参与者包括 40 名对照者和 31 名 PFD 患者，其原因是：非综合征性致密颗粒 (DG) 缺乏症 (PFD-DGD，n = 9)、RUNX1 单倍体不足 (n = 6) 和其他不明原因引起的聚集缺陷 (n = 16)。 TG 用 PRP 和 PPP 样品进行了测试。由于 DG 储存的 ADP 和多磷酸盐可增强血小板依赖性 TG，因此对 PFD-DGD PRP TG 进行了测试，以使用 ADP、多磷酸盐和组合添加剂进行校正。还评估了组织因子途径抑制剂 (TFPI)、血小板因子 V (FV) 以及血小板 TFPI 和 ANO6 转录物水平。测试结果与 TG 终点和出血的关联。 PFD 样本的 PRP TG 受损，但 PPP TG 也受损，其 PRP 和 PPP TG 端点之间具有很强的相关性 (P ≤ .005)。 PFD-DGD PRP TG 终点显示与 PPP TG 终点相关，但与 DG 计数无关，并且通过添加聚磷酸盐和激动剂得到改善，但未完全纠正。 PFD 参与者的血浆 TFPI 升高，血小板 TFPI 降低 (P≤.02)，但血小板 FV、血小板 TFPI 和 ANO6 转录物水平正常。 PFD 血浆 TFPI 水平与多个 PPP TG 终点显着相关 (P ≤ .04)。几个 PFD PRP TG 终点显示与出血症状显着相关，包括伤口愈合问题和小割伤导致的长时间出血 (P ≤ .04)。 TG 在常见的 PFD 中受损，他们的 PRP TG 结果显示出与症状的有趣关联。
Studies of thrombin generation (TG) with platelet‐rich plasma (PRP) and platelet‐poor plasma (PPP) have provided insights on bleeding disorders. We studied TG for a cohort with commonly encountered platelet function disorders (PFD). Participants included 40 controls and 31 with PFD due to: nonsyndromic dense granule (DG) deficiency (PFD‐DGD, n = 9), RUNX1 haploinsufficiency (n = 6) and aggregation defects from other, uncharacterized causes (n = 16). TG was tested with PRP and PPP samples. As DG store ADP and polyphosphate that enhance platelet‐dependent TG, PFD‐DGD PRP TG was tested for correction with ADP, polyphosphate and combined additives. Tissue factor pathway inhibitor (TFPI), platelet factor V (FV), and platelet TFPI and ANO6 transcript levels were also evaluated. Findings were tested for associations with TG endpoints and bleeding. PFD samples had impaired PRP TG, but also impaired PPP TG, with strong associations between their PRP and PPP TG endpoints (P ≤ .005). PFD‐DGD PRP TG endpoints showed associations to PPP TG endpoints but not to DG counts, and were improved, but not fully corrected, by adding polyphosphate and agonists. PFD participants had increased plasma TFPI and reduced platelet TFPI (P ≤ .02) but normal levels of platelet FV, and platelet TFPI and ANO6 transcripts levels. PFD plasma TFPI levels showed significant association to several PPP TG endpoints (P ≤ .04). Several PFD PRP TG endpoints showed significant associations to bleeding symptoms, including wound healing problems and prolonged bleeding from minor cuts (P ≤ .04). TG is impaired in commonly encountered PFD, with their PRP TG findings showing interesting associations to symptoms.
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