Girolami A, Scarparo P, Bonamigo E, Treleani M, Lombardi A M
Department of Medical and Surgical Sciences and Northeastern Italy Association for the Study of Coagulation Disorders, University of Padua Medical School, Padua, Italy.
Hematology. 2012 Nov;17(6):350-4. doi: 10.1179/1024533212Z.000000000144.
The reagents most frequently used for FVII activity assay are obtained by rabbit brain or human placenta. In recent years, human recombinant thromboplastins have received great attention. FVII activity in FVII deficiency is usually low, regardless of the thromboplastin used. There are a few exceptions to this rule. These are represented by FVII Padua (Arg304Gln), FVII Nagoya (Arg304Trp), and FVII (Arg79Gln). In these three instances, clear discrepancies were noted in the FVII activity depending on the thromboplastin used. This indicates that at least two areas of FVII are involved in tissue binding, namely an epidermal growth factor domain of the light chain (Arg79Gln) and the catalytic domain (Arg304), controlled by exons 4 and 8, respectively. Since these three variants are cross reactive material positive, namely they are Type 2 defects, all other variants with normal antigen should be investigated by a panel of at least three tissue thromboplastins (rabbit brain, human tissue or human recombinant, and ox brain derived) in order to obtain a satisfactory classification.
用于FVII活性测定的最常用试剂是从兔脑或人胎盘中获得的。近年来，重组人凝血活酶受到了广泛关注。无论使用何种凝血活酶，FVII缺乏症患者的FVII活性通常都较低。但也有一些例外情况。这些例外包括FVII帕多瓦（Arg304Gln）、FVII名古屋（Arg304Trp）和FVII（Arg79Gln）。在这三种情况下，根据所使用的凝血活酶，FVII活性存在明显差异。这表明FVII至少有两个区域参与组织结合，即轻链的表皮生长因子结构域（Arg79Gln）和催化结构域（Arg304），分别由外显子4和8控制。由于这三种变异体交叉反应物质呈阳性，即它们属于2型缺陷，因此对于所有其他抗原正常的变异体，应使用至少三种组织凝血活酶（兔脑、人组织或重组人凝血活酶以及牛脑来源的凝血活酶）进行检测，以便获得满意的分类结果。
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