Liu Chen, Han Man, Zhao Lei, Zhu Mengjie, Xu Qinzhu, Song Ying, Wang Hui
a Department of Clinical Laboratory , Peking University People's Hospital , Beijing , People's Republic of China.
Hematology. 2019 Dec;24(1):129-133. doi: 10.1080/10245332.2018.1532648. Epub 2018 Oct 16.
This research aimed to explore the significance of low activity of ADAMTS-13 in acute myeloid leukemia (AML) after bone morrow transplantation (BMT), and to evaluate the disease progress and prognosis of the patients with low or normal activity of ADAMTS-13 after BMT.
46 AML patients were included in our research. ADAMTS-13 activity was measured before BMT. Their medical indicators were recorded one month after BMT. All the patients were followed up and their disease progression was evaluated afterwards. The medical indicators and prognosis situation were compared between Low ADAMTS13 Group (<481 ng/ml) and Normal Group (481-785 ng/ml) according to the reference range of local laboratory. ROC curves were used to evaluate the predictive value of ADAMTS13 for infection complications and survival.
Low ADAMTS13 Group show extended APTT, PT and elevated CRP and D-Dimer levels, compared with Normal Group. Low ADAMTS13 Group suffered more BMT-related complications than Normal Group. In addition, Low ADAMTS13 Group underwent higher mortality than Normal Group in the one-year follow up after BMT and two-year follow up after onset of AML. ADAMTS13 has AUC of 0.7675, 0.7254, 0.8019 for lung infection, CMV infection and death within one year after BMT, suggesting that ADAMTS13 has predictive value for prognosis of AML patients after BMT.
For patients with low ADAMTS13 activity, the prognosis was worse and the probability of serious complications and mortality was significantly higher than AML patients with normal ADAMTS13 activity, which suggest predictive role of ADAMTS13 activity for the prognosis of AML after BMT.
AML patients with low activity of ADAMTS-13 had worse prognosis after BMT.
本研究旨在探讨急性髓系白血病（AML）患者骨髓移植（BMT）后ADAMTS-13活性降低的意义，并评估BMT后ADAMTS-13活性低或正常的患者的疾病进展及预后。
本研究纳入46例AML患者。在BMT前检测ADAMTS-13活性。BMT后1个月记录其医学指标。对所有患者进行随访并评估其疾病进展。根据当地实验室的参考范围，比较ADAMTS13低活性组（<481 ng/ml）和正常组（481 - 785 ng/ml）的医学指标及预后情况。采用ROC曲线评估ADAMTS13对感染并发症及生存的预测价值。
与正常组相比，ADAMTS13低活性组的活化部分凝血活酶时间（APTT）、凝血酶原时间（PT）延长，C反应蛋白（CRP）和D-二聚体水平升高。ADAMTS13低活性组比正常组发生更多与BMT相关的并发症。此外，在BMT后1年随访及AML发病后2年随访中，ADAMTS13低活性组的死亡率高于正常组。ADAMTS13对BMT后1年内肺部感染、巨细胞病毒（CMV）感染及死亡的曲线下面积（AUC）分别为0.7675、0.7254、0.8019，提示ADAMTS13对BMT后AML患者的预后有预测价值。
ADAMTS13活性低的患者预后较差，严重并发症及死亡的概率显著高于ADAMTS13活性正常的AML患者，这提示ADAMTS13活性对BMT后AML的预后有预测作用。
ADAMTS-13活性低的AML患者BMT后的预后较差。
