Rapid ADAMTS13 availability impacts treatment for microangiopathic hemolytic anemia and thrombocytopenia
Rapid ADAMTS13 availability impacts treatment for microangiopathic hemolytic anemia and thrombocytopenia
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DOI：
10.1002/jca.21419
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发表时间：
2016-10-01
影响因子：
1.5
通讯作者：
Dunbar, Nancy M.
中科院分区：
文献类型：
--
作者：
Martin, Isabella W.;Katus, Matthew C.;Dunbar, Nancy M.
血栓性血小板减少性紫癜（TTP）可表现为一系列临床表现。当TTP在患者的临床鉴别诊断中时，应紧急启动治疗性血浆置换（TPE）。具有血小板反应蛋白1型基序的A型去整合素和金属蛋白酶，成员13（ADAMTS 13）的酶活性水平与不断发展的临床表现相结合，可以指导进一步治疗，包括TPE的持续时间和频率以及液体置换的选择。我们更换参考实验室以获得更快的ADAMTS 13活性水平周转时间的经验导致临床管理发生显著变化，包括总体TPE程序减少，以及在无ADAMTS 13严重缺乏和TTP临床怀疑指数较低的患者中偶尔使用白蛋白作为部分置换液。临床单采31：419-422，2016。(c)2015 Wiley Periodicals，Inc.
Thrombotic thrombocytopenic purpura (TTP) can present with a spectrum of clinical manifestations. When TTP is in a patient's clinical differential diagnosis, therapeutic plasma exchange (TPE) should be initiated emergently. Enzyme activity level of A Disintegrin And Metalloproteinase with a Thrombospondin type 1 motif, member 13 (ADAMTS13) in conjunction with the evolving clinical picture can guide further therapy, including duration and frequency of TPE and choice of fluid replacement. Our experience switching reference laboratories to obtain a more rapid turnaround time of ADAMTS13 activity level resulted in significant changes in clinical management, including fewer overall TPE procedures and the occasional use of albumin for a portion of the replacement fluid in patients without severe deficiency of ADAMTS13 and a low index of clinical suspicion for TTP. J. Clin. Apheresis 31:419-422, 2016. (c) 2015 Wiley Periodicals, Inc.