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干燥综合征相关的血栓性血小板减少性紫癜的临床分析 被引量：1

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摘要 血栓性血小板减少性紫癜（TTP）是累及多器官系统的血栓性微血管病,先天性TTP是由于血管性血友病因子裂解蛋白酶（vWF-CP）基因杂合子或纯合子的复合突变,为常染色体隐性遗传,呈复发趋向。获得性TTP包括特发性（占33%-57%）和继发性（占43%-66%）[1],后者常继发于自身免疫性疾病、妊娠、感染、药物、恶性肿瘤或造血干细胞移植等。
作者 孙幸福 卢瑞南 陆化
机构地区 南京市高淳区人民医院血液病科 南京医科大学第一附属医院血液病科
出处 《江苏医药》 CAS 北大核心 2014年第17期2086-2088,共3页 Jiangsu Medical Journal
基金 江苏省中医药局科技项目(L213219)
关键词 血栓性血小板减少性紫癜 干燥综合征 血管性血友病因子裂解蛋白酶 临床分析 常染色体隐性遗传 血栓性微血管病 自身免疫性疾病 造血干细胞移植
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10

  • 1王学文.获得性血栓性血小板减少性紫癜免疫治疗的进展[J].中国微循环,2007,11(3):209-212. 被引量：2
  • 2Toumeh A,Josh N,Narwal R,et al.Refractory thrombotic thrombocytopenic purpura associated with primary Sjgren′s syndrome treated with Rituximab:a case report[J].Am J Ther,2014,21(2):e56-e60.
  • 3Schattner A,Friedman J,Klepfish A,et al.Thrombotic thrombocytopenic purpura as an initial presentation of primary Sjgren′s syndrome[J].Clin Rheumatol,2002,21(1):57-59.
  • 4Abe H,Tsuboi N,Yukawa S,et al.Thrombotic thrombocytopenic purpura complicating Sjgren′s syndrome with crescentic glomerulonephritis and membranous nephritis[J].Mod Rheumatol,2004,14(2):174-178.
  • 5Koga T,Yamasaki S,Nakamura H,et al.Renal thrombotic microangiopathies/thrombotic thrombocytopenic purpura in a patient with primary Sjgren′s syndrome complicated with IgM monoclonal gammopathy of undetermined significance[J].Rheumatol Int,2013,33(1):227-230.
  • 6Lin TY,Chang CC,Chang CC,et al.Cyclophosphamiderescued plasmapheresis-unresponsive secondary thrombotic thrombocytopenic purpura caused by Sjgren′s syndrome[J].Arch Med Sci,2012,8(5):934-938.
  • 7Yamashita H,Takahashi Y,Kaneko H,et al.Thrombotic thrombocytopenic purpura with an autoantibody to ADAMTS13complicating Sjgren′s syndrome:two cases and a literature review[J].Mod Rheumatol,2013,23(2):365-373.
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二级参考文献20

  • 1藤村吉博.TTPと HUS[J].医学のぁゅみ,2003,206(1):101-104.
  • 2Vesely SK,George JN,Lammle B,et al.ADAMTS-13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome:related to presenting features and clinical outcomes in a prospective cohort of 142 patients[J].Blood,2003,102(1):60 ～68
  • 3Kremer Hovinga JA,Studt JD,Alberio L,et al.vonWillebrand factor-Cleaving protease (ADAMTS-13) activity determination in the diagnosis of thrombotic microangiopathies:The Swiss experience[J].Semin Hematol,2004,41 (1):75 ～ 82
  • 4Gutterman LA,Kloster B,Tsai HM.Rituximab therapy for refractory thrombotic thrombocytopenic purpura[J].Blood Cells Mod Dis,2000,28(3):385 ～391
  • 5Tsai HM,Shulman K.Rituximab induces remission of cerebral ischemia caused by thrombotic thrombocytopenic purpura[J].Eur J Haematol,2003,70 (3):183 ～ 185
  • 6Sallah S,Husain A,Wan JY,et al.Rituximab in patients with refractory thrombotic thrombocytopenic purpura[J].J Thromb Haemost,2004,2:834 ～ 836
  • 7Reddy PS,Deauna-Limayo D,Cook JD,et al.Rituximab in the treatment of relapsed thrombotic thrombocytopenic purpura[J].Ann Hematol,2005,84 (2):232 ～ 235
  • 8Fakhouri F,Vernant JP,Veyradier A,et al.Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura:a study of 11 cases[J].Blood,2005,106(6):1932 ～ 1937
  • 9Allan DS,Kovacs MJ,Clark WF.Frequently relapsing thrombotic thrombocytopenic purpura treated with cytotoxic immunosuppressive therapy[J].Haematologica,2001,86 (8):844 ～ 850
  • 10Dervenoulas J,Tsirigotis P,Bollas G,et al.Effeicacy of intravascular immunoglobulin in the treatment of thrombotic thrombocytopenic purpura[J].A study of 44 cases,2001,105(4):204 ～205

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江苏医药
2014年 第17期

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