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特发性血小板减少性紫癜(ITP)中血小板功能与血小板计数无关,且具有时间一致性,并与当前和后续出血严重程度相关。

Platelet Function in ITP, Independent of Platelet Count, Is Consistent Over Time and Is Associated with Both Current and Subsequent Bleeding Severity.

机构信息

Center for Platelet Research Studies, Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts, United States.

Division of Hematology/Oncology, Boston Children's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts, United States.

出版信息

Thromb Haemost. 2018 Jan;118(1):143-151. doi: 10.1160/TH17-06-0387. Epub 2018 Jan 5.

Abstract

BACKGROUND

Treatment decisions for patients with immune thrombocytopenia (ITP) are difficult because patients with similarly low platelet counts differ in their bleeding tendency. We recently reported that platelet function tests, independent of platelet count, are associated with bleeding severity, suggesting that these tests may be useful indicators of future bleeding in ITP.

OBJECTIVES

To test this hypothesis, we evaluated the consistency of these platelet function tests over time and their association with bleeding severity.

METHODS

Bleeding score and platelet biomarkers were evaluated in a cross-sectional study of children with ITP at two visits separated by a median of 10 months.

RESULTS AND CONCLUSIONS

Correlations between Visit 1 and Visit 2 results for immature platelet fraction, circulating and agonist-stimulated platelet surface P-selectin, and activated GPIIb-IIIa and GPIbα indicated consistency of the platelet phenotype over time. Consistent with our previous findings, platelet biomarkers at each visit were significantly associated with the concurrent bleeding score. Furthermore, increased P-selectin on circulating platelets and reduced agonist-stimulated P-selectin and activated GPIIb-IIIa-positive platelets at Visit 1 were significantly associated with bleeding scores at Visit 2 and remained significantly associated with bleeding severity after adjustment for platelet count. These results suggest a mechanistic link between desensitization of agonist receptors and increased bleeding severity. In summary, platelet function in ITP, independent of platelet count, is consistent over time and is associated with both concurrent and subsequent bleeding severity. These findings support further evaluation of platelet function testing to help guide patient management in ITP.

摘要

背景

对于免疫性血小板减少症(ITP)患者的治疗决策较为困难,因为血小板计数相近的患者出血倾向存在差异。我们最近报道,血小板功能测试独立于血小板计数,与出血严重程度相关,这表明这些测试可能是 ITP 患者未来出血的有用指标。

目的

为了验证这一假说,我们评估了这些血小板功能测试随时间的一致性及其与出血严重程度的相关性。

方法

采用前瞻性队列研究,在 10 个月的中位数时间间隔内,对 2 次就诊的 ITP 患儿进行出血评分和血小板生物标志物评估。

结果与结论

初次就诊和再次就诊时的幼稚血小板分数、循环和激动剂刺激的血小板表面 P 选择素、激活的 GpIIb-IIIa 和 GPIbα之间的相关性表明,血小板表型随时间具有一致性。与我们之前的发现一致,每次就诊的血小板生物标志物与同期出血评分显著相关。此外,初次就诊时循环血小板上 P-选择素增加、激动剂刺激的 P-选择素和激活的 GpIIb-IIIa 阳性血小板减少与二次就诊时的出血评分显著相关,且在调整血小板计数后仍与出血严重程度显著相关。这些结果提示激动剂受体脱敏与出血严重程度增加之间存在机制联系。总之,ITP 患者的血小板功能独立于血小板计数,随时间具有一致性,且与同期和后续出血严重程度相关。这些发现支持进一步评估血小板功能测试,以帮助指导 ITP 患者的管理。

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