Recombinant ADAMTS 13 in thrombotic thrombocytopenic purpura
نویسندگان
چکیده
منابع مشابه
A case of Acquired Thrombotic Thrombocytopenic Purpura without Renal Dysfunction
Objective: Thrombotic thrombocytopenic purpura (TTP) is the most malignant variant of microangiopathy that usually presents by typical symptoms including thrombocytopenia, hemolytic anemia, neurological abnormalities, fever and renal impairment. Report of the Case: We report a 12-year-old male presented by cytopenia, fever, purpura on his extremities, seizure and lethargy. Peripheral blood sm...
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Thrombotic thrombocytopenic purpura (TTP) is mostly attributed to the presence of an autoantibody against ADAMTS-13, a metalloprotease that degrades ultralarge von Willebrand protein multimers. Accumulation of vWF multimers and systemic platelet aggregation lead to microangiopathic thrombosis, hemolytic anemia, and end-organ ischemia. Most patients respond to therapeutic plasma exchange (TPE), ...
متن کاملThe Interaction between Factor H and Von Willebrand Factor
Complement factor H (fH) is a plasma protein that regulates activation of the alternative pathway, and mutations in fH are associated with a rare form of thrombotic microangiopathy (TMA), known as atypical hemolytic uremic syndrome (aHUS). A more common TMA is thrombotic thrombocytopenic purpura, which is caused by the lack of normal ADAMTS-13-mediated cleavage of von Willebrand factor (VWF). W...
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BACKGROUND AND OBJECTIVES Acquired thrombotic thrombocytopenic purpura (TTP) is often due to autoantibodies inhibiting ADAMTS-13 activity resulting in impaired processing of very large von Willebrand factor multimers. TTP usually presents with an acute onset and a fulminant, sometimes fatal course. With appropriate treatment including plasma exchange, and fresh frozen plasma replacement, often ...
متن کاملSuccessful treatment of thrombotic thrombocytopenic purpura during pregnancy: A case report
A 28 years’ pregnant woman with 24 weeks’ gestational age referred with petechiae and purpura from previous day without any trauma. She had an occipital headache from last night. Overt petechial and purpuric lesions were seen in the mouth and skin. There was neither hepatosplenomegaly nor lymphadenopathy. She was conscious and oriented. The patient was febrile, anemic and thromboc...
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عنوان ژورنال:
دوره 4 شماره
صفحات -
تاریخ انتشار 2017