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一例获得性短暂性出血倾向与获得性血小板贮存池缺陷和血栓素 A2 生成缺陷相关。

A case of acquired transient bleeding diathesis associated with acquired platelet storage pool deficiency and defective thromboxane A2 production.

机构信息

Laboratorio di Emostasi e Trombosi - Dipartimento di Scienze della Salute, Università degli Studi di Milano, Milano, Italy.

Struttura Complessa di Medicina Generale II, Ospedale San Paolo, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milano, Italy.

出版信息

Platelets. 2024 Dec;35(1):2358241. doi: 10.1080/09537104.2024.2358241. Epub 2024 Jun 4.

DOI:10.1080/09537104.2024.2358241
PMID:38832819
Abstract

Acquired disorders of platelet function are an underdiagnosed cause of bleeding tendency. A 14-year-old girl developed moderate mucocutaneous bleeding two weeks after a infection successfully treated with clarithromycin. The patient was referred to us 7 months later for laboratory investigation of the persisting bleeding diathesis. The patient's personal and family histories were negative for bleeding disorders. Complete blood count, von Willebrand Factor levels and coagulation tests were normal; platelet aggregation, ATP secretion, δ-granules content and serum thromboxane B2 levels were defective. At follow-up visits, laboratory parameters and the bleeding diathesis progressively normalized within 2 years. The patient's condition is compatible with a diagnosis of acquired Storage Pool Deficiency (SPD), associated with defective thromboxane A2 production. To our knowledge, this is the first case of acquired, transient SPD with spontaneous remission. The pathogenic role of infection or clarithromycin is possible, albeit uncertain.

摘要

获得性血小板功能障碍是出血倾向的一种未被充分诊断的原因。一名 14 岁女孩在成功用克拉霉素治疗后,两周后出现中度黏膜皮肤出血。7 个月后,患者因持续存在的出血倾向被转介到我们这里进行实验室检查。患者的个人和家族史均无出血性疾病。全血细胞计数、血管性血友病因子水平和凝血试验均正常;血小板聚集、ATP 分泌、δ-颗粒含量和血清血栓素 B2 水平存在缺陷。在随访期间,2 年内实验室参数和出血倾向逐渐恢复正常。患者的病情符合获得性储存池缺陷(SPD)的诊断,伴有血栓烷 A2 产生缺陷。据我们所知,这是首例自发缓解的获得性、短暂性 SPD 病例。感染或克拉霉素的致病作用可能存在,但不确定。

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