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获得性储存池缺陷伴血小板相关IgG升高。5例报告。

Acquired storage pool deficiency with increased platelet-associated IgG. Report of five cases.

作者信息

Weiss H J, Rosove M H, Lages B A, Kaplan K L

出版信息

Am J Med. 1980 Nov;69(5):711-7. doi: 10.1016/0002-9343(80)90436-2.

Abstract

Acquired abnormalities of platelet aggregation have been reported with increasing frequency. We studied five patients (including two with systemic lupus erythematosus and one with compensated chronic idiopathic thrombocytopenic purpura) in whom platelet aggregation responses to collagen, epinephrine and ADP are impaired; in all cases, we found that levels of platelet-associated immunoglobulin G (IgG) were increased. In all five patients substances stored in platelet-dense granules (ATP, ADP, serotonin and calcium) were diminished. The content of the alpha-granule substance, beta-thromboglobulin, was also decreased in most cases, whereas the levels of two secretable acid hydrolase enzymes (beta-glucuronidase and beta-N-acetyl glucosaminidase) were within normal limits. These findings are similar to those observed in subtypes of congenital storage pool deficiency. However, in contrast to the congenital disorder, a membrane-bound (nonsecretable) acid phosphatase was also decreased in the patients with acquired storage pool deficiency. These findings suggest that impaired platelet aggregation on an acquired basis may, in some patients, be due to immune platelet damage resulting in a distinctive type of platelet storage pool deficiency.

摘要

获得性血小板聚集异常的报道日益增多。我们研究了5例患者(包括2例系统性红斑狼疮患者和1例代偿性慢性特发性血小板减少性紫癜患者),这些患者对胶原、肾上腺素和二磷酸腺苷(ADP)的血小板聚集反应受损;在所有病例中,我们发现血小板相关免疫球蛋白G(IgG)水平升高。所有5例患者血小板致密颗粒中储存的物质(三磷酸腺苷(ATP)、ADP、5-羟色胺和钙)均减少。大多数病例中α颗粒物质β-血小板球蛋白的含量也降低,而两种可分泌酸性水解酶(β-葡萄糖醛酸酶和β-N-乙酰氨基葡萄糖苷酶)的水平在正常范围内。这些发现与先天性储存池缺乏症亚型中观察到的情况相似。然而,与先天性疾病不同的是,获得性储存池缺乏症患者中一种膜结合(不可分泌)酸性磷酸酶也降低。这些发现表明,在某些患者中,获得性血小板聚集受损可能是由于免疫性血小板损伤导致一种独特类型的血小板储存池缺乏症。

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