Casonato A, Fabris F, Boscaro M, Girolami A
Blut. 1987 May;54(5):281-8. doi: 10.1007/BF00320876.
Factor VIII/von Willebrand factor (VIII/vWf) related properties were studied in twenty six patients with thrombocytopenia. Fifteen patients were affected by idiopathic thrombocytopenic purpura (ITP) and 11 patients by thrombocytopenia of a different nature or non-ITP (n-ITP). All patients showed an enhancement of platelet associated IgG (PAIgG). A significant increase of factor VIII ristocetin cofactor (VIII R: RCoF) and factor VIII related antigen (VIII R:Ag) was found in ITP patients while normal values were observed for factor VIII coagulant (VIII:C). All factor VIII/vWf components, on the contrary, were increased in n-ITP group with a prevalence of VIII R:RCoF as observed in ITP group even though with lower mean values. Multimeric analysis of VIII/vWf demonstrated a higher concentration of all multimeric components, with major representation of higher molecular weight multimers (HMWM) in patients of both groups. Two patients were studied before and after improvement in platelet count. A decrease of vWf related properties (VIII R:RCoF and VIII R:Ag) concomitant with the increase in platelet count was found. In n-ITP patients a statistical correlation between VIII R:RCoF and PAIgG was also observed while no correlation was found between other factor VIII/vWf components and PAIgG both in ITP and n-ITP patients.
对26例血小板减少症患者的凝血因子VIII/血管性血友病因子（VIII/vWf）相关特性进行了研究。15例患者患有特发性血小板减少性紫癜（ITP），11例患者患有其他性质的血小板减少症或非ITP（n - ITP）。所有患者血小板相关IgG（PAIgG）均升高。ITP患者中凝血因子VIII瑞斯托霉素辅因子（VIII R:RCoF）和凝血因子VIII相关抗原（VIII R:Ag）显著增加，而凝血因子VIII促凝活性（VIII:C）值正常。相反，n - ITP组中所有VIII/vWf成分均增加，VIII R:RCoF占优势，与ITP组情况相同，尽管平均值较低。对VIII/vWf进行多聚体分析显示，两组患者所有多聚体成分浓度均较高，高分子量多聚体（HMWM）占主要部分。对2例患者在血小板计数改善前后进行了研究。发现随着血小板计数增加，vWf相关特性（VIII R:RCoF和VIII R:Ag）降低。在n - ITP患者中，还观察到VIII R:RCoF与PAIgG之间存在统计学相关性，而在ITP和n - ITP患者中，其他VIII/vWf成分与PAIgG之间均未发现相关性。
