Girolami A, Sticchi A, Burul A, Zanon R D
Vox Sang. 1977;32(4):230-8. doi: 10.1111/j.1423-0410.1977.tb00635.x.
23 patients with hemophilia B have been investigated by means of several immunological methods. 16 patients (69.9%) had no detectable factor XI antigen. Five had a normal factor IX antigen and the electrophoretic mobility of this abnormal factor IX was similar to that of its normal counterpart. One of these five patients had hemophilia Bm, since ox brain thromboplastin clotting time was severely prolonged. The remaining two patients had reduced or decreased factor IX antigen. Several patients showed a slight protongation of ox brain thromboplastin time due to an associated slight factor VII deficiency. On the basis of these results, a tentative classification of hemophilia B into five variants is proposed, namely: hemctor IX antigen; hemophilia Bra, or with reduced factor IX antigen; hemophilia Bm, or with normal factor IX antigen and severely prolonged ox brain thromboplastin; hemophilia B patients is feasible only by means of a battery of tests, namely:factor IX activity assay, factor IX antigen determination, ox brain thromboplastin clotting time, factor VII activity assay.
通过多种免疫学方法对23例B型血友病患者进行了研究。16例患者（69.9%）未检测到因子XI抗原。5例患者因子IX抗原正常，且这种异常因子IX的电泳迁移率与其正常对应物相似。这5例患者中有1例患有Bm型血友病，因为牛脑凝血活酶凝血时间严重延长。其余2例患者因子IX抗原减少或降低。由于合并轻度因子VII缺乏，部分患者牛脑凝血活酶时间略有延长。基于这些结果，提出了将B型血友病初步分为五个变体，即：因子IX抗原缺乏型；Bra型血友病，或因子IX抗原减少型；Bm型血友病，或因子IX抗原正常且牛脑凝血活酶严重延长型；B型血友病患者只有通过一系列检测才能明确，即：因子IX活性测定、因子IX抗原测定、牛脑凝血活酶凝血时间、因子VII活性测定。
