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本文引用的文献

1
Poor responder to plasma exchange therapy in acquired thrombotic thrombocytopenic purpura is associated with ADAMTS13 inhibitor boosting: visualization of an ADAMTS13 inhibitor complex and its proteolytic clearance from plasma.获得性血栓性血小板减少性紫癜患者对血浆置换治疗反应不佳与ADAMTS13抑制剂增强有关：ADAMTS13抑制剂复合物的可视化及其从血浆中的蛋白水解清除。
Transfusion. 2015 Oct;55(10):2321-30. doi: 10.1111/trf.13182. Epub 2015 Jun 8.
2
ADAMTS13 deficiency in severe postpartum HELLP syndrome.严重产后HELLP综合征中的ADAMTS13缺乏症。
Br J Haematol. 2013 Nov;163(3):409-10. doi: 10.1111/bjh.12494. Epub 2013 Jul 24.
3
ADAMTS13 unbound to larger von Willebrand factor multimers in cryosupernatant: implications for selection of plasma preparations for thrombotic thrombocytopenic purpura treatment.在冷冻上清液中与较大的 von Willebrand 因子多聚体分离的 ADAMTS13：对选择用于治疗血栓性血小板减少性紫癜的血浆制剂的影响。
Transfusion. 2013 Dec;53(12):3192-202. doi: 10.1111/trf.12182. Epub 2013 Apr 8.
4
Pathogenesis of preeclampsia.子痫前期的发病机制。
Annu Rev Pathol. 2010;5:173-92. doi: 10.1146/annurev-pathol-121808-102149.
5
Acute activation of the endothelium results in increased levels of active von Willebrand factor in hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome.内皮细胞的急性激活导致溶血、肝酶升高和血小板减少（HELLP）综合征中活性血管性血友病因子水平升高。
J Thromb Haemost. 2006 Dec;4(12):2569-75. doi: 10.1111/j.1538-7836.2006.02205.x. Epub 2006 Sep 12.
6
Novel monoclonal antibody-based enzyme immunoassay for determining plasma levels of ADAMTS13 activity.用于测定血浆中ADAMTS13活性水平的新型基于单克隆抗体的酶免疫测定法。
Transfusion. 2006 Aug;46(8):1444-52. doi: 10.1111/j.1537-2995.2006.00914.x.
7
Factor VIII and von Willebrand factor changes during normal pregnancy and puerperium.正常妊娠和产褥期因子VIII及血管性血友病因子的变化。
Blood Coagul Fibrinolysis. 2003 Oct;14(7):647-51. doi: 10.1097/00001721-200310000-00005.
8
Mild to moderate reduction of a von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome.患有HELLP微血管病综合征的孕妇中血管性血友病因子裂解蛋白酶（ADAMTS - 13）轻度至中度降低。
Haematologica. 2003 Sep;88(9):1029-34.
9
Procoagulant and anticoagulant mechanisms in human placenta.人类胎盘的促凝血和抗凝血机制。
Semin Thromb Hemost. 2003 Apr;29(2):175-84. doi: 10.1055/s-2003-38833.
10
Ten years of prophylactic treatment with fresh-frozen plasma in a child with chronic relapsing thrombotic thrombocytopenic purpura as a result of a congenital deficiency of von Willebrand factor-cleaving protease.因先天性血管性血友病因子裂解蛋白酶缺乏导致慢性复发性血栓性血小板减少性紫癜的患儿接受十年新鲜冰冻血浆预防性治疗。
Br J Haematol. 2001 Jun;113(3):649-51. doi: 10.1046/j.1365-2141.2001.02808.x.

HELLP综合征患者血浆中与血管性血友病因子未结合的游离形式ADAMTS13严重减少。

Severe reduction of free-form ADAMTS13, unbound to von Willebrand factor, in plasma of patients with HELLP syndrome.

作者信息

Yoshida Yoko, Matsumoto Masanori, Yagi Hideo, Isonishi Ayami, Sakai Kazuya, Hayakawa Masaki, Hori Yuji, Sado Toshiyuki, Kobayashi Hiroshi, Fujimura Yoshihiro

机构信息

Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan.

Japanese Red Cross Kinki Block Blood Center, Ibaraki, Japan; and.

出版信息

Blood Adv. 2017 Aug 23;1(20):1628-1631. doi: 10.1182/bloodadvances.2017006767. eCollection 2017 Sep 12.

DOI:10.1182/bloodadvances.2017006767
PMID:29296808
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5728335/
Abstract

Severely decreased ADAMTS13 unbound to VWF may play a key role in the pathogenesis of HELLP syndrome.A qualitative ADAMTS13 assay may be important for diagnosing HELLP syndrome.

摘要

与血管性血友病因子（VWF）未结合的ADAMTS13严重减少可能在HELLP综合征的发病机制中起关键作用。ADAMTS13定性检测对诊断HELLP综合征可能很重要。