Treliński Jacek, Pachniewska Katarzyna, Matczak Justyna, Robak Marta, Chojnowski Krzysztof
Department of Hematology, Medical University of Łódź, Poland.
Department of Hemostasis, Medical University of Łódź, Poland.
Adv Clin Exp Med. 2016 Nov-Dec;25(6):1255-1263. doi: 10.17219/acem/65781.
Congenital fibrinogen disorders (CFD) are rare fibrinogen deficiencies which may be quantitative or functional. The clinical course of hypofibrinogenemia (hypoFI) or dysfibrinogenemia (dysFI) is unpredictable and cannot be determined by the application of standard hemostasis tests.
The main aim of this study was to assess ROTEM parameters in CFD patients.
Nine patients with CFD were studied. The fibrinogen concentration was measured functionally and antigenically. EXTEM, INTEM, FIBTEM and APTEM tests were used to measure selected ROTEM parameters, including maximum clot firmness (MCF). Fibrin plasma polymerization, clot lysis and plasmin amidolytic activity were determined by spectrophotometric methods.
Incorporating the antigenic, ELISA method, to the diagnostic workup allowed the initial diagnosis to be switched from hypoFI to dysFI in 3/7 patients. MCF readings (the most important parameter describing fibrin polymerization capacity) were significantly lower in patients than in controls according to all ROTEM tests. Cases with hypoFI demonstrated markedly lower readings of MCF according to all ROTEM tests than cases with dysFI. All patients demonstrated disturbances of fibrin polymerization process assessed by turbidimetry. In contrast, no marked differences were identified between studied groups in reference to plasmin amidolytic activity.
Our data suggests that ROTEM and fibrin plasma polymerization according to the turbidimetric method have a high sensitivity towards detection of different CFD. Although ROTEM MCF assessment may help discriminate patients with hypoor dysfibrinogenemia, this finding has to be confirmed on larger groups of patients.
先天性纤维蛋白原异常（CFD）是罕见的纤维蛋白原缺乏症，可能是定量性或功能性的。低纤维蛋白原血症（hypoFI）或异常纤维蛋白原血症（dysFI）的临床病程不可预测，无法通过标准止血测试来确定。
本研究的主要目的是评估CFD患者的旋转血栓弹力图（ROTEM）参数。
对9例CFD患者进行了研究。通过功能法和抗原法测量纤维蛋白原浓度。使用EXTEM、INTEM、FIBTEM和APTEM测试来测量选定的ROTEM参数，包括最大血凝块硬度（MCF）。通过分光光度法测定纤维蛋白血浆聚合、凝块溶解和纤溶酶酰胺水解活性。
在诊断检查中采用抗原性ELISA方法，使3/7的患者初始诊断从hypoFI转变为dysFI。根据所有ROTEM测试，患者的MCF读数（描述纤维蛋白聚合能力的最重要参数）显著低于对照组。根据所有ROTEM测试，hypoFI患者的MCF读数明显低于dysFI患者。通过比浊法评估，所有患者均表现出纤维蛋白聚合过程的紊乱。相比之下，研究组之间在纤溶酶酰胺水解活性方面未发现明显差异。
我们的数据表明，ROTEM和比浊法测定的纤维蛋白血浆聚合对检测不同的CFD具有高敏感性。尽管ROTEM MCF评估可能有助于区分低纤维蛋白原血症或异常纤维蛋白原血症患者，但这一发现必须在更大规模的患者群体中得到证实。
