Inherited thrombotic thrombocytopenic purpura
نویسندگان
چکیده
منابع مشابه
Inherited thrombotic thrombocytopenic purpura in pregnancy.
OBJECTIVE The primary pathologic reason for thrombotic thrombocytopenic purpura (TTP) lies in the systemic formation of platelet aggregations in association with endothelial cells damage. Endothelial damage is a result of an abnormal synthesis and metabolism of unusually large von Willebrand Factor (ULvWF) multimers. In normal conditions vWF cleaving metalloprotease, known as ADAMTS-13 (A Disin...
متن کاملInherited thrombotic thrombocytopenic purpura mimicking immune thrombocytopenic purpura during pregnancy: a case report
BACKGROUND Thrombotic thrombocytopenic purpura is a very rare hereditary blood deficiency disorder of ADAMTS13 (von Willebrand factor-cleaving protease) and a life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. The deficiency in ADAMTS13 metalloprotease, which cleaves the von Willebrand factor, may be congenital or acquired. The c...
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 کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Haematologica
سال: 2009
ISSN: 0390-6078,1592-8721
DOI: 10.3324/haematol.2008.002493