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首页> 外文期刊>Blood: The Journal of the American Society of Hematology >Functional platelet defects in children with severe chronic ITP as tested with 2 novel assays applicable for low platelet counts.
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Functional platelet defects in children with severe chronic ITP as tested with 2 novel assays applicable for low platelet counts.

机译：用2种新型测定的严重慢性ITP患儿的功能血小板缺陷，适用于低血小板计数。

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摘要

Immune thrombocytopenia (ITP) is an autoimmune disease with a complex heterogeneous pathogenesis and a bleeding phenotype that is not necessarily correlated to platelet count. In this study, the platelet function was assessed in a well-defined cohort of 33 pediatric chronic ITP patients. Because regular platelet function test cannot be performed in patients with low platelet counts, 2 new assays were developed to determine platelet function: first, the microaggregation test, measuring in platelets isolated from 10 mL of whole blood the platelet potential to form microaggregates in response to an agonist; second, the platelet reactivity assay, measuring platelet reactivity to adenosine diphosphate (ADP), convulxin (CVX), and thrombin receptor activator peptide in only 150 μL of unprocessed whole blood. Patients with a severe bleeding phenotype demonstrated a decreased aggregation potential upon phorbol myristate acetate stimulation, decreased platelet degranulation following ADP stimulation, and a higher concentration of ADP and CVX needed to activate the glycoprotein IIbIIIa complex compared with patients with a mild bleeding phenotype. In conclusion, here we have established 2 functional tests that allow for evaluation of platelet function in patients with extremely low platelet counts (<10(9)). These tests show that platelet function is related to bleeding phenotype in chronic ITP.
机译：免疫血小板减少症（ITP）是一种自身免疫性疾病，具有复杂的异质性发病机制和出血表型，不一定与血小板计数相关。在该研究中，血小板函数在明确定义的33例小儿慢性ITP患者中评估。由于血小板计数患者不能进行常规血小板函数测试，开发了2种新测定以确定血小板功能：首先，微见试验，测量从10ml全血中分离的血小板势力响应形成微烧结。激动剂;二，血小板反应性测定，测量血小板反应性与腺苷二磷酸（ADP），催化剂（CVX）和凝血酶受体活化剂肽中仅为150μL未加工的全血。具有严重出血表型的患者表现出脂肪栓腹膜醋酸酯刺激的血压潜力下降，ADP刺激后的血小板脱溶，以及与温和出血表型的患者相比，激活糖蛋白IIIBIIIA复合物所需的更高浓度的ADP和CVX。总之，在这里，我们已经建立了2个功能试验，可用于评估血小板计数极低患者的血小板功能（<10（9））。这些测试表明，血小板功能与慢性ITP中的出血表型有关。

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