Song Jaewoo, Lee Kyung A, Park Tae Sung, Park Rojin, Choi Jong Rak
Department of Laboratory Medicine, Yonsei University College of Medicine, and Soon Chun Hyang University Hospital, 250 Seongsanno, Seodaemun-gu, Seoul, Korea 120-752.
Ann Clin Lab Sci. 2008 Autumn;38(4):368-75.
Von Willebrand factor (VWF) cleaving metalloprotease, ADAMTS13, known for its causative relation to thrombotic thrombocytopenic purpura (TTP), also decreases to variable degree in other clinical conditions associated with thrombocytopenia, indicating a possible contribution of moderate deficiency of ADAMTS13 to platelet dynamics. We measured ADAMTS13 activity along with VWF activity, collagen binding activity (VWF:CB), and thrombin/antithrombin complex (TAT) in plasma drawn from patients with consumptive coagulopathy, in whom the platelet count was closely followed. ADAMTS13 activity was significantly but variably decreased in the patients, and VWF activity and VWF:CB were markedly increased as expected. The platelet count itself was not correlated with ADAMTS13 activity, VWF activity, or VWF:CB. However, the rate of decline of log-scaled platelet count (DeltaLnPLT/day) correlated well with ADAMTS13 activity and VWF:CB. ADADMTS13 activity showed inverse correlation with VWF:CB. Moreover, the correlation between ADAMTS13 and DeltaLnPLT/day was preserved even after VWF:CB was controlled. Multiple regression analysis showed that ADAMTS13 activity was the sole factor explaining DeltaLnPLT/day among ADAMTS13, VWF:CB, TAT, prothrombin time, d-dimer, and fibrinogen. TAT level and d-dimer as indicators of systemic fibrinolytic activity did not correlate with ADAMTS13 activity. In conclusion, we found that the decrease of ADADMTS13 activity in consumptive coagulopathy has stronger relationship to platelet dynamics than has generally been recognized.
血管性血友病因子（VWF）裂解金属蛋白酶ADAMTS13因与血栓性血小板减少性紫癜（TTP）存在因果关系而闻名，在其他与血小板减少相关的临床病症中，它也会有不同程度的降低，这表明ADAMTS13中度缺乏可能对血小板动力学有一定影响。我们检测了消耗性凝血病患者血浆中的ADAMTS13活性以及VWF活性、胶原结合活性（VWF:CB）和凝血酶/抗凝血酶复合物（TAT），这些患者的血小板计数受到密切监测。患者的ADAMTS13活性显著降低，但存在差异，VWF活性和VWF:CB如预期显著升高。血小板计数本身与ADAMTS13活性、VWF活性或VWF:CB无相关性。然而，对数标度的血小板计数下降率（DeltaLnPLT/天）与ADAMTS13活性和VWF:CB密切相关。ADAMTS13活性与VWF:CB呈负相关。此外，即使在控制了VWF:CB后，ADAMTS13与DeltaLnPLT/天之间的相关性依然存在。多元回归分析表明，在ADAMTS13、VWF:CB、TAT、凝血酶原时间、D-二聚体和纤维蛋白原中，ADAMTS13活性是解释DeltaLnPLT/天的唯一因素。作为全身纤溶活性指标的TAT水平和D-二聚体与ADAMTS13活性无相关性。总之，我们发现消耗性凝血病中ADAMTS13活性的降低与血小板动力学的关系比一般认为的更为密切。
