Thrombotic thrombocytopenic purpura: pathogenesis, diagnosis and potential novel therapeutics

Summary Thrombotic thrombocytopenic purpura (TTP), a potentially fatal clinical syndrome, is primarily caused by autoantibodies against the von Willebrand factor (VWF)‐cleaving metalloprotease ADAMTS‐13. In general, severe deficiency of plasma ADAMTS‐13 activity ( 10 IU dL−1) in a similar clinical c...

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Veröffentlicht in:	Journal of thrombosis and haemostasis 2017-10, Vol.15 (10), p.1889-1900
Hauptverfasser:	Saha, M., McDaniel, J. K., Zheng, X. L.
Format:	Artikel
Sprache:	eng
Schlagworte:	ADAMTS13 Protein - blood ADAMTS13 Protein - genetics ADAMTS13 Protein - immunology ADAMTS13 Protein - therapeutic use Anemia Anemia, Hemolytic - blood Anemia, Hemolytic - diagnosis Anemia, Hemolytic - therapy Antagonists Autoantibodies Autoantibodies - blood Corticosteroids Cyclophosphamide Cyclosporins Diagnosis Diagnosis, Differential Gene therapy Genetic Therapy Haptoglobin Hemoglobin Hemolytic anemia Hemolytic uremic syndrome Hemostasis Humans Immunomodulation Immunotherapy L-Lactate dehydrogenase Lactic acid Metalloproteinase Monoclonal antibodies Morbidity Pathogenesis pathology Plasma Plasma Exchange Platelets Predictive Value of Tests Prophylaxis Purpura Purpura, Thrombotic Thrombocytopenic - blood Purpura, Thrombotic Thrombocytopenic - diagnosis Purpura, Thrombotic Thrombocytopenic - epidemiology Purpura, Thrombotic Thrombocytopenic - therapy Recombinant Proteins - therapeutic use Remission Risk Factors Rituximab Splenectomy therapeutics Thrombocytopenia Thrombocytopenia - blood Thrombocytopenia - diagnosis Thrombocytopenia - therapy Thrombocytopenic purpura thrombotic microangiopathies Thrombotic microangiopathy Treatment Outcome von Willebrand factor
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