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血小板功能障碍中间综合征

Intermediate syndrome of platelet dysfunction.

作者信息

Czapek E E, Deykin D, Salzman E, Lian E C, Hellerstein L J, Rosoff C B

出版信息

Blood. 1978 Jul;52(1):103-13.

PMID:656620
Abstract

We studied eight patients with intermittent bleeding episodes usually following trauma and associated with the ingestion of medicine known to interfere with platelet function. All patients had a normal or minimally prolonged baseline bleeding time. All had a normal platelet count, glass bead retention test, and platelet serotonin content and a variable pattern of abnormalities in prothrombin consumption and platelet factor 3 availability. However, all showed abnormal platelet aggregation reactions using epinephrine, adenosine diphosphate, and collagen. Following the administration of 975 mg aspirin, our patients' bleeding times became prolonged to a greater extent than the bleeding times of normal controls (range 13 to greater than 20 min). Review of the literature showed approximately 5% of "normal" controls had findings similar to those we report. We believe we are describing a group of individuals with an intermediate form of platelet dysfunction. Although their bleeding diathesis is not as severe as that of patients with platelet dysfunction syndromes previoulsy described, they do bleed significantly when subjected to trauma following the ingestion of drugs such as aspirin. We propose that this defect is common and should be screened for. The aspirin tolerance test is a simple test for detecting these patients.

摘要

我们研究了8例患者,这些患者通常在创伤后出现间歇性出血发作,并与摄入已知会干扰血小板功能的药物有关。所有患者的基线出血时间正常或仅轻微延长。所有患者的血小板计数、玻璃珠滞留试验、血小板血清素含量均正常,凝血酶原消耗和血小板因子3活性存在不同模式的异常。然而,所有患者使用肾上腺素、二磷酸腺苷和胶原进行血小板聚集反应均显示异常。给予975毫克阿司匹林后,我们患者的出血时间延长程度大于正常对照组(范围为13至超过20分钟)。文献回顾显示,约5%的“正常”对照有与我们报告相似的结果。我们认为我们正在描述一组具有中间形式血小板功能障碍的个体。虽然他们的出血素质不如先前描述的血小板功能障碍综合征患者严重,但在摄入阿司匹林等药物后遭受创伤时,他们确实会大量出血。我们建议这种缺陷很常见,应该进行筛查。阿司匹林耐量试验是检测这些患者的一种简单试验。

相似文献

1
Intermediate syndrome of platelet dysfunction.血小板功能障碍中间综合征
Blood. 1978 Jul;52(1):103-13.
2
[Aspirin-like defect - a hereditary thrombocytopathy due to impaired release of platelet adenosine diphosphate].[阿司匹林样缺陷——一种因血小板二磷酸腺苷释放受损导致的遗传性血小板病]
Bilt Hematol Transfuz. 1979;7(2-3):165-74.
3
Spontaneous hyphema associated with ingestion of aspirin and ethanol.
Am J Ophthalmol. 1976 Oct;82(4):631-4. doi: 10.1016/0002-9394(76)90553-5.
4
Clinico-haematological profile of isolated PF3 availability defect: therapeutic potential of soya bean--a pilot study.
Eur J Haematol. 1999 May;62(5):327-31. doi: 10.1111/j.1600-0609.1999.tb01910.x.
5
A study of aspirin induced changes in bleeding time, platelet aggregation, and Sonoclot coagulation analysis in humans.一项关于阿司匹林对人体出血时间、血小板聚集及Sonoclot凝血分析影响的研究。
Ann Clin Lab Sci. 1991 Sep-Oct;21(5):315-27.
6
Congenital release thrombocytopathy: pathophysiology and management.先天性释放血小板病:病理生理学与管理
Obstet Gynecol. 1976 Oct;48(4):403-6.
7
A syndrome of factor VII deficiency and abnormal platelet release reaction.一种因子 VII 缺乏与异常血小板释放反应综合征。
Scand J Haematol. 1978 Sep;21(3):206-14. doi: 10.1111/j.1600-0609.1978.tb00355.x.
8
Platelet dysfunction in homozygous beta-thalassemia.纯合子β地中海贫血中的血小板功能障碍
Pediatr Res. 1979 Dec;13(12):1345-9. doi: 10.1203/00006450-197912000-00009.
9
Abnormalities in platelet function due to defects in the release reaction.
Ann N Y Acad Sci. 1972 Oct 27;201:161-73. doi: 10.1111/j.1749-6632.1972.tb16297.x.
10
Transient, spontaneous bruising with long bleeding time and normal platelet count.短暂性、自发性瘀斑,出血时间延长而血小板计数正常。
Am J Clin Pathol. 1975 Jan;63(1):81-6. doi: 10.1093/ajcp/63.3.81.

引用本文的文献

1
Interaction between the LMWH reviparin and aspirin in healthy volunteers.低分子量肝素瑞肝素与阿司匹林在健康志愿者中的相互作用。
Br J Clin Pharmacol. 2000 Apr;49(4):337-41. doi: 10.1046/j.1365-2125.2000.00173.x.
2
Disorders of platelet function: mechanisms, diagnosis and management.血小板功能障碍:机制、诊断与管理
West J Med. 1981 Feb;134(2):109-27.