Hereditary thrombotic thrombocytopenic purpura
نویسندگان
چکیده
منابع مشابه
Hereditary thrombotic thrombocytopenic purpura and the hereditary TTP registry.
Hereditary thrombotic thrombocytopenic purpura, Upshaw-Schulman syndrome, ADAMTS13 Hereditary thrombotic thrombocytopenic purpura (TTP), also known as Upshaw-Schulman syndrome, is a rare recessively inherited disease. Underlying is a severe constitutional deficiency of the von Willebrand factor-cleaving protease, ADAMTS13, due to compound heterozygous or homozygous mutations in the ADAMTS13 gen...
متن کاملThrombotic Thrombocytopenic Purpura
Thrombotic microangiopathy comprises a spectrum of clinical and lab findings including microangiopathic hemolytic anemia, thrombocytopenia, and thrombosis of capillary and arterioles. Platelet and hyaline thrombi with complete or partial occlusion of these vessels are integral histopathological findings. These findings are seen irrespective of cause and organ involved. Pathogenesis and prognosi...
متن کاملThrombotic thrombocytopenic purpura.
A 6-year-old boy presented with microangiopathic hemolytic anemia, thrombo-cytopenia, altered sensorium and intractable bleeding. A diagnosis of thrombotic thrombocytopenic purpura was made and the child recovered dramatically after plasmapheresis. Recent developments in the understanding of TTP are reviewed, including the importance of a metaloprotease required to cleave multimers of von Willi...
متن کاملThrombotic Thrombocytopenic Purpura: Diagnosis and Treatment
Thrombotic Thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathic disease, associated with thrombocytopenia and hemolytic anemia. It is caused by an enzymatic dysfunction responsible in cleavage of blood clotting factors. In this study we have tried to review the available approaches in diagnoses of the disease as well as treatment strategies. Based on the what the current review ...
متن کاملPlatelets: thrombotic thrombocytopenic purpura.
Abnormalities of plasma von Willebrand factor (VWF) have been recognized to be associated with thrombotic thrombocytopenic purpura (TTP) for over 20 years. Patients with chronic, relapsing TTP have VWF multimers that are larger than normal, similar in size to those secreted by cultured endothelial cells. Recent observations have documented that a deficiency of a VWF-cleaving protease (termed AD...
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ژورنال
عنوان ژورنال: Haematologica
سال: 2019
ISSN: 0390-6078,1592-8721
DOI: 10.3324/haematol.2019.225896