一名原发性血小板增多症患儿血小板对经典激动剂的不应性。
Platelet refractoriness to classical agonists in a child with essential thrombocythemia.
作者信息
Jaime-Perez Jose Carlos, Gomez-Almaguer David
机构信息
Autonomous University of Nuevo Leon, Monterrey, Mexico.
出版信息
Platelets. 2005 Feb;16(1):61-2. doi: 10.1080/09537100400007424.
BACKGROUND
Essential thrombocythemia is a rare disease during childhood. Platelet morphological abnormalities are frequent and defects in platelet function tests, mainly hypoaggregation, occur.
MATERIALS AND METHODS
An incidental diagnosis of essential thrombocythemia was established in a 9-year-old boy with a platelet count of 2050 x 10(9)/l. His platelets were studied for aggregation defects with four classical agonists employing optical aggregometry.
RESULTS
Aggregation ranged from 5% for adrenaline, 8% for collagen, 12% for ristocetin, to 25% with adenosine diphosphate, followed by complete disaggregation.
CONCLUSION
Platelet refractoriness to classical agonists, probably compounded by platelet GPIb deficiency, was documented. The differential diagnosis is discussed.
背景
原发性血小板增多症是儿童期罕见疾病。血小板形态异常常见，且血小板功能测试存在缺陷，主要表现为聚集功能减退。
材料与方法
一名9岁男孩血小板计数为2050×10⁹/L，偶然诊断为原发性血小板增多症。采用光学聚集法，使用四种经典激动剂研究其血小板聚集缺陷。
结果
肾上腺素诱导的聚集率为5%，胶原诱导的为8%，瑞斯托霉素诱导的为12%，二磷酸腺苷诱导的为25%，随后完全解聚。
结论
记录到血小板对经典激动剂的抵抗，可能因血小板糖蛋白Ib缺乏而加重。讨论了鉴别诊断。
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