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血栓性血小板减少性紫癜研究进展 被引量：1

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摘要 血栓性血小板减少性紫癜（TTP）是一种罕见且严重的血栓性微血管病,以发热、血小板减少、微血管性溶血性贫血、神经系统症状和肾脏损害五联征为典型临床表现。前三大特征称为三联征。本病最早由Moschowitz描述,多年来五联征一直作为主要诊断依据[1]。大多数TTP起病急骤、病情凶险,如不及时治疗,病死率高达90%。
作者 伏杭江 黄先华 马元
机构地区 南京军区南京总医院干部保健科 南京医科大学第一附属医院急救中心
出处 《人民军医》 2014年第12期1359-1361,共3页 People's Military Surgeon
关键词 血栓性血小板减少性紫癜 血浆置换 研究进展
分类号 R554.6 [医药卫生—血液循环系统疾病]
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参考文献15

  • 1Zhon Z. VonWillebrand factor, ADAMTS B, and thrombotic Thrombocytopenic purpura[J]. Semin Thromb Hemost, 2010,36 ( 1 ) : 71- 81.
  • 2Furlan M, Robles R, Lamie B,et al. Partia 1 purification and charac- terization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis[J]. Blood, 1996, 87(10) :4223 -4234.
  • 3Sadler JE. Biochemistry and genetics of yon Willebrand factor [J]. Annu Rev Biochem,1998,67(4) :395 -424.
  • 4Banno F, Kokame K, Okuda T, et al. Complete deficiency in AD- AMTS13 is prothrombotic, but it alone is not suffident to cause thrombotic thrombocytopenic purpura [J ]. Blood, 2006, 107 ( 8 ) 3161-3166.
  • 5Shelat SG,Smith AG, Ai J, et al. Inhibitory autoantibodies against ADAMTSB in patients with thrombotic thrombocytopenic purpura bind ADAMTS13 protease and may accelerate its clearance in vivo [J]. J Thromb Haemost,2006,4(8) :1707-1717.
  • 6Studt JD, Kremer Hovinga JA, Radonic R, et al. Familial acquired thrombotic thrombocytopenic purpura: ADAMTS 13 inhibitory au- toantboclies in identical twins[J]. Blood,2004,103(11):4195-4197.
  • 7George JN. Clinical practice: Thrombotic thrombocytopenic purpura [J]. N Eng 1 J Med,2006,354(18):1927-1935.
  • 8Moake JL Thrombotic microangiopathies[J]. N Engl J Med, 2002, 347(8) :589-600.
  • 9Fontana S, Kremer Hovinga JA, Studt JD, et al. Plasma therapy in thrombotic thrombocytopenic purpura Review of the literature and the Bern expexienee in a subgroup of patients with severe acquired ADAMTS13 deficiency[J]. Semin Hematol, 2004,41 (1) : 48-59.
  • 10Rock G,Clark WF,Anderson D,et al. ADAMTS 13 may not predict disease or outcome in patients with Thrombotic Thrombocytopenic Purpura[J]. Thromb Res, 2013,131 (4) : 308 -312.

二级参考文献28

  • 1Zhou Z. Von Willebrand factor, ADAMTS 13, and thrombotic Thrombocytopenic purpura [J]. Semin Thromb Hemost, 2010, 36 (1): 71-81.
  • 2Moake J. Thrombotic microangiopatbies: multimers, metalloprotease, and beyond [J]. Clin & Trans Sci, 2009, 2 (5): 366-373.
  • 3Gurkan E, Baslamisli F, Guvenc B, et al. Thrombotic tlrrombocytopenic purpura in southern Turkey: a single-center experience of 29 cases [J]. Clin lab Haemato, zos, Tl (2): 121-125.
  • 4George IN, Terrell DR, Swisher KK, et al. Lessons learned from the Oklahoma thrombotic thrombocytopenic purpura-hemolytic uremic syndrome registry [J]. J Clin Apheresis , 2008, 23 (4): 129-137.
  • 5Furlan M. Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura [J]. Exp Rev Card Ther, 2003,1 (2): 243-255.
  • 6Sadler JE. von Willebrand factor in its native environment [J]. Blood, 2013, 121 (14): 2583-2584.
  • 7Shah N, Rutherford C, Matevosyan K, et al. Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TIP) [J]. Brit J Haem, 2013, 163: 514-519.
  • 8Shelat SG, Smith AG, AI J, et al. Inhibitory autoantibodies against ADAMTS13 in patients with thrombotic thrombocytopenic purpura bind ADAMTS13 protease and may accelerate its clearance in vivo [J]. Thromb Haemost, 2006, 4 (8): 1707-1717.
  • 9Reese J, Darrshini A, Muthurajah S, et al. Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired adamts13 deficiency: comparison of incidence, demographic and clinical features [J]. Pediatr Blood Cancer, 2013,60 (10): 1676-1682.
  • 10Nguyen TC, Han YY. Plasma exchange therapy for thrombotic microangiopathies [J]. Organogenesis, 2011 , 7 (1): 28-31.

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人民军医
2014年 第12期

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