Thrombotic Thrombocytopenic Purpura: A Thrombotic Disorder Caused by ADAMTS13 Deficiency
نویسندگان
چکیده
منابع مشابه
Deficiency of ADAMTS13 and thrombotic thrombocytopenic purpura.
Thrombotic thrombocytopenic purpura, characterized by the presence of systemic hyaline thrombi in the arterioles and capillaries, is a potentially fatal disease that responds to plasma infusion or exchange. Recent studies have demonstrated that a metalloprotease in the normal plasma cleaves endothelial von Willebrand factor to a series of multimers. A deficiency of the protease, due to autoimmu...
متن کاملDeficiency of ADAMTS13 Causes Thrombotic Thrombocytopenic Purpura
In the circulation, a plasma metalloprotease, ADAMTS13, cleaves von Willebrand factor (vWF) in a shear-dependent manner. This article reviews the role of this cleavage in regulating VWF-platelet interaction and proposes a scheme for understanding how a deficiency of ADAMTS13 results in the development of microthrombi in patients with thrombotic thrombocytopenic purpura. (Arterioscler Thromb Vas...
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Thrombotic thrombocytopenic purpura (TTP) is a hematological disease characterized by microangiopathic hemolytic anemia and thrombocytopenia. Although the link between ADAMTS13 deficiency and idiopathic TTP has been well-established, the role of trimethoprim-sulfamethoxazole (TMP-SMX) in the pathogenesis of TTP is not yet well elucidated. To the best of our knowledge, there have been only two p...
متن کاملThrombotic thrombocytopenic purpura: The role of ADAMTS13.
Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disease requiring prompt diagnosis and initiation of therapeutic plasma exchange to improve patient survival. However, diagnosis is often difficult because of atypical presentations and signs and symptoms that resemble other conditions. Measurements of ADAMTS13 activity, ADAMTS13 inhibitor, and ADAMTS13 autoantibody are ...
متن کاملADAMTS13, Thrombotic Thrombocytopenic Purpura and Pregnancy
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) which pathophysiology mainly relies on a severe deficiency (either acquired or inherited) of ADAMTS13, the specific von Willebrand factor (VWF) protease. TTP is characterized by a feminine predominance and pregnancy is a precipitating factor for TTP boots. Obstetrical TTP represents at least 20% of all TTP occurring...
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ژورنال
عنوان ژورنال: Hematology/Oncology Clinics of North America
سال: 2007
ISSN: 0889-8588
DOI: 10.1016/j.hoc.2007.06.003