Current opinion in hematology
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Thrombotic thrombocytopenic purpura (TTP) has been a mysterious and deadly disease that often could be treated effectively by plasma exchange, but without real understanding of the underlying pathophysiology. Recent advances now suggest that deficiency of a specific von Willebrand factor (VWF) cleaving protease promotes tissue injury in TTP. VWF multimers participate in the formation of platelet thrombi. ⋯ The VWF cleaving protease proves to be a new member of the ADAMTS family of metalloproteases, designated ADAMTS13. Autoantibodies that inhibit ADAMTS13 cause sporadic TTP, and mutations in the ADAMTS13 gene cause an autosomal recessive form of chronic relapsing TTP. Further studies of ADAMTS13 seem likely to change our approach to the diagnosis and treatment of TTP and other thrombotic microangiopathies.
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The ability to use an effective treatment constitutes a main concern in the management of acute ischemic stroke. Randomized trials have so far failed to demonstrate any beneficial effect of neuroprotective agents on neurologic outcome after an acute stroke. ⋯ Among them, a single randomized trial, the National Institute of Neurological Diseases and Stroke Trial, showed a beneficial effect of thrombolysis on outcome after an acute ischemic stroke with a significant increase in the number of patients with no or minimal disability at 3 months in the group receiving intravenous recombinant tissue plasminogen activator (rt-PA) compared with placebo. However, there was no reduction in mortality and there was, as in all other trials, a significant increase in the risk of symptomatic intracerebral hemorrhage (ICH).