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1例血友病伴抑制剂患者的换血疗法和脉冲式类固醇治疗

Exchange transfusion and pulse steroid therapy in a hemophiliac with inhibitor.

作者信息

Chuansumrit A, Isarangkura P, Krutvecho T, Hathirat P, Pintadit P

出版信息

J Med Assoc Thai. 1989 Jan;72 Suppl 1:139-43.

PMID:2499649
Abstract

In Thailand, the management of bleeding in hemophiliacs with inhibitor is still a challenging aspect. The source of Factor VIII is locally prepared fresh frozen plasma and cryoprecipitate. The Factor VIII concentrate and other commercial blood products are not available because of the remarkably high price. Successful management of bleeding in a 3-year-old hemophiliac boy with moderate inhibitor level (11.82 BU) was reported. He had active bleeding from a 1 cm size cut wound at upper gingivo-buccal fold and did not respond to local measures and supportive treatment. He received exchange transfusion, intravenous cyclophosphamide and high dosage of cryoprecipitate. The bleeding stopped and the inhibitor declined to 2.6 BU. A week later, the bleeding recurred with an anamnestic response of inhibitor to 5 BU. The second exchange transfusion and methylprednisolone as pulse steroid therapy were given daily for 3 days. No cryoprecipitate was infused. The bleeding gradually stopped within 48 hours and the inhibitor level declined to 3.2 BU which was still at low level for at least 2 months. Exchange transfusion and methylprednisolone as pulse steroid therapy may be an alternative treatment for controlling bleeding in a hemophiliac with moderate inhibitor level in countries where a high concentrate of Factor VIII or other blood products are not available. It is practical, simple, effective and of low cost.

摘要

在泰国,对患有抑制物的血友病患者进行出血管理仍是一个具有挑战性的方面。凝血因子VIII的来源是当地制备的新鲜冰冻血浆和冷沉淀。由于价格极高,凝血因子VIII浓缩物和其他商业血液制品无法获得。有报道称,一名3岁的血友病男孩,抑制物水平中等(11.82 BU),其出血得到了成功管理。他在上牙龈颊皱襞处有一个1厘米大小的切割伤口并伴有活动性出血,局部措施和支持性治疗均无效。他接受了换血、静脉注射环磷酰胺和高剂量冷沉淀治疗。出血停止,抑制物水平降至2.6 BU。一周后,出血复发,抑制物出现回忆反应至5 BU。进行了第二次换血,并给予甲基强的松龙进行为期3天的脉冲类固醇治疗。未输注冷沉淀。出血在48小时内逐渐停止,抑制物水平降至3.2 BU,至少在2个月内仍处于低水平。在无法获得高浓缩凝血因子VIII或其他血液制品的国家,换血和甲基强的松龙脉冲类固醇疗法可能是控制中等抑制物水平血友病患者出血的替代治疗方法。它实用、简单、有效且成本低。

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