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[输血后紫癜。6个月内在同一家医院确诊的2例病例描述]

[Post-transfusion purpura. Description of 2 cases diagnosed at the same hospital in a 6-month period].

作者信息

Muñiz-Díaz E, Sandoval V, de la Calle O, Cecchini C, Gil S, Puig L, Madoz P

机构信息

Servicio de Hemoterapia, Hospital de Sant Pau, Universitat Autónoma de Barcelona.

出版信息

Sangre (Barc). 1996 Feb;41(1):59-64.

PMID:8779037
Abstract

Two new cases of postransfusional purpura diagnosed at the same hospital within the space of six months are described. This finding supports the idea that despite being an infrequent disorder, a substantial rise in PTP cases inside and outside our country, has been recorded. This increase has coincided with a greater interest in platelet immunology and, particularly, in complications associated with blood transfusion. Both cases constitute an example of the clinical epidemiological profile which characterizes the patients suffering from this disorder. The patients are two women aged 74 and 60 years who after 8 and 9 days, respectively, of being transfused with red cells developed a severe thrombocytopenia accompanied by generalized haemorrhagic diathesis. The serological studies performed revealed the presence, in both patients, of an HPA-1a platelet specific antibody. The platelet genotyping enabled us to confirm this specificity after detecting an HPA-1 (a-b+) platelet genotype. The treatment with immunoglobulins at high doses proved to be effective in both cases. The adsorption-elution experiments of the antibody versus HPA-1 (a + b) platelets were positive in the patient with the highest antibody titre (1024). This finding support the most recent hypothesis concerning the pathogenic mechanism of PTP. According to this theory, the antibody, which is detected in the acute phase of the PTP, would not yet have acquired the restricted specificity corresponding to it. This could enable it to react with a structure shared by the HPA-1a positive and HPA-1a negative platelets.

摘要

本文描述了在同一家医院6个月内确诊的两例输血后紫癜新病例。这一发现支持了这样一种观点,即尽管该疾病并不常见,但国内外已记录到PTP病例大幅增加。这种增加与对血小板免疫学,特别是与输血相关并发症的更大关注相吻合。这两个病例构成了患有这种疾病患者的临床流行病学特征实例。患者为两名女性,年龄分别为74岁和60岁,在输注红细胞8天和9天后,分别出现严重血小板减少症并伴有全身出血倾向。所进行的血清学研究显示,两名患者均存在HPA-1a血小板特异性抗体。血小板基因分型在检测到HPA-1(a-b+)血小板基因型后,使我们能够确认这种特异性。高剂量免疫球蛋白治疗在两例中均被证明有效。抗体与HPA-1(a + b)血小板的吸附-洗脱实验在抗体滴度最高(1024)的患者中呈阳性。这一发现支持了关于PTP致病机制的最新假说。根据这一理论,在PTP急性期检测到的抗体尚未获得与其对应的受限特异性。这可能使其能够与HPA-1a阳性和HPA-1a阴性血小板共有的结构发生反应。

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