Furlan Miha
Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland.
Expert Rev Cardiovasc Ther. 2003 Jul;1(2):243-55. doi: 10.1586/14779072.1.2.243.
Thrombotic thrombocytopenic purpura (TTP) is a dramatic intravascular platelet-clumping disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, renal insufficiency and fever. TTP is a rare disease but is almost always fatal if untreated. More than 80% of patients survive with plasma therapy. In healthy individuals, the proteolytic cleavage of ultralarge von Willebrand factor (vWF) multimers prevents spontaneous clumping of platelets in the microcirculation. Patients with TIP have either severe congenital deficiency of von Willebrand factor-cleaving protease (vWF-cp), or have autoantibodies that inhibit the protease. Determination of vWF-cp levels in patient plasma helps to distinguish between TTP and other thrombotic microangiopathies with similar clinical signs and symptoms. vWF-cp is a member of the ADAMTS family of metalloproteases and has been designated ADAMTS13.
血栓性血小板减少性紫癜（TTP）是一种严重的血管内血小板聚集紊乱疾病，其特征为微血管病性溶血性贫血、血小板减少、神经异常、肾功能不全和发热。TTP是一种罕见疾病，但如果不治疗几乎总是致命的。超过80%的患者通过血浆疗法存活。在健康个体中，超大血管性血友病因子（vWF）多聚体的蛋白水解切割可防止血小板在微循环中自发聚集。TIP患者要么严重先天性缺乏血管性血友病因子裂解蛋白酶（vWF-cp），要么具有抑制该蛋白酶的自身抗体。测定患者血浆中的vWF-cp水平有助于区分TTP与其他具有相似临床体征和症状的血栓性微血管病。vWF-cp是金属蛋白酶ADAMTS家族的成员，已被命名为ADAMTS13。
