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[对蛋白A柱有反应的难治性特发性血小板减少性紫癜]

[Refractory idiopathic thrombocytopenic purpura which responded to protein A column].

作者信息

Tauchi T, Suzuki A, Fujimura T, Iwabuchi H, Ohyashiki J H, Ohyashiki K, Toyama K, Nagasawa H

出版信息

Rinsho Ketsueki. 1989 Aug;30(8):1289-93.

PMID:2689685
Abstract

Currently, there is no satisfactory therapy available for patients with chronic idiopathic thrombocytopenic purpura (ITP) who are unresponsive to conventional therapeutic modalities. In this report, we describe a patient with chronic refractory ITP treated with immobilized protein A in an extracorporeal system. The patient was a 74 years old male diagnosed as ITP in 1980. Despite steroid therapy, the disease progressed and the patient exhibited ecchymosis and gum bleeding which was unresponsive to intravenous gammaglobulin therapy. Severe gastrointestinal bleeding was evident and administration of danazol was discontinued due to liver dysfunction. The patient was treated with extracorporeal protein A immunoabsorption. The patient's whole blood (200-300 ml per treatment) was separated into plasma and cellular components and the plasma was passed through an immunoabsorption column containing 200 mg of covalently bound protein A. The treated plasma and cellular components were returned to the patient. After 4 immunoabsorption treatments, the platelet counts elevated and there was evidence of improvement in gastrointestinal bleeding. This report indicates that protein A immunoabsorption therapy should be considered in patients with chronic refractory ITP.

摘要

目前,对于那些对传统治疗方式无反应的慢性特发性血小板减少性紫癜(ITP)患者,尚无令人满意的治疗方法。在本报告中,我们描述了一名慢性难治性ITP患者,其在体外系统中接受了固定化蛋白A治疗。该患者为一名74岁男性,于1980年被诊断为ITP。尽管接受了类固醇治疗,但病情仍进展,患者出现瘀斑和牙龈出血,对静脉注射丙种球蛋白治疗无反应。严重的胃肠道出血明显,且因肝功能障碍停用了达那唑。该患者接受了体外蛋白A免疫吸附治疗。患者的全血(每次治疗200 - 300毫升)被分离成血浆和细胞成分,血浆通过含有200毫克共价结合蛋白A的免疫吸附柱。处理后的血浆和细胞成分回输给患者。经过4次免疫吸附治疗后,血小板计数升高,胃肠道出血有改善迹象。本报告表明,对于慢性难治性ITP患者应考虑蛋白A免疫吸附治疗。

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