Coincident classic hemophilia and "idiopathic" thrombocytopenic purpura in patients under treatment with concentrates of antihemophilic factor (factor VIII).
Coincident classic hemophilia and "idiopathic" thrombocytopenic purpura in patients under treatment with concentrates of antihemophilic factor (factor VIII).
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在接受浓缩抗血友病因子（因子 VIII）治疗的患者中，同时出现经典血友病和“特发性”血小板减少性紫癜。
DOI：
10.1056/nejm198302243080808
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发表时间：
1983
期刊：
影响因子：
--
通讯作者：
Lederman,MM
中科院分区：
文献类型：
--
作者：
Ratnoff,OD;Menitove,JE;Aster,RH;Lederman,MM
现在广泛采用冻干浓缩抗血友病因子（因子 VIII）在家治疗经典血友病。我们对五名正在接受此类治疗的严重经典血友病患者进行了类似慢性特发性血小板减少性紫癜的综合征研究。表 1 至表 3 总结了实验室研究。血小板相关 IgG 含量增加、四名泼尼松治疗患者的良好反应以及一名患者脾切除后病情缓解，所有这些都表明患者的疾病类似于特发性血小板减少性紫癜。此外，对四名患者的研究表明，三名患者的细胞介导免疫受损。病例报告患者 1A，21 岁。 。 。
Home treatment of classic hemophilia with lyophilized concentrates of antihemophilic factor (factor VIII) is now widely practiced. We studied a syndrome resembling chronic idiopathic thrombocytopenic purpura in five patients with severe classic hemophilia who were receiving such therapy. Laboratory studies are summarized in Tables1through3. The presence of increased amounts of platelet-associated IgG, the favorable response in four patients treated with prednisone, and remission after splenectomy in one patient all suggest that the patients' disorder resembled idiopathic thrombocytopenic purpura. In addition, studies in four patients demonstrated evidence of impaired cell-mediated immunity in three.Case ReportsPatient 1A 21-year-old . . .