A case of refractory thrombotic thrombocytopenic purpura treated with plasmapheresis and rituximab.
نویسندگان
چکیده
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder with no prevalence or incidence studies in sub-Saharan Africa. Acquired TTP has several causes, all of which lead to decreased activity of von Willebrand factor cleaving protease (ADAMTS13) due to autoantibodies that are directed towards ADAMTS13. We report a case of a 46-year-old man who presented with most of the classic clinical manifestations of TTP.
منابع مشابه
Successful Treatment of Refractory Thrombotic Thrombocytopenic Purpura with Rituximab, a Monoclonal Antibody: a Case Report
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), ...
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عنوان ژورنال:
- South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde
دوره 106 7 شماره
صفحات -
تاریخ انتشار 2016