Current opinion in hematology
-
New treatment modalities have become increasingly popular for the treatment of acute thrombotic thrombocytopenic purpura. Widespread availability of ADAMTS13 assays resulted in the increased recognition of patients with hereditary thrombotic thrombocytopenic purpura and specific issues related to acquired ADAMTS13 deficiency. These new aspects with implications on management of thrombotic thrombocytopenic purpura patients are reviewed here. ⋯ Despite progress in understanding the pathophysiology of thrombotic thrombocytopenic purpura, acute bouts as well as relapses still represent serious health threats to patients and rapid initiation of plasma exchange is mandatory. Large randomized clinical trials, however, need to determine whether new treatment modalities are superior to standard plasma exchange.
-
An impaired function of the protein C pathway plays a central role in the pathogenesis of sepsis. Administration of human recombinant activated protein C (Xigris) may restore the dysfunctional anticoagulant mechanism and may simultaneously modulate the pro-inflammatory response. Initial clinical studies with activated protein C in patients with sepsis showed a reduction of 28-day mortality. However, subsequent studies did cast some doubt on the efficacy and also the safety of this treatment. ⋯ Clinical studies support the use of activated protein C in patients with severe sepsis; however, in view of the substantial skepticism surrounding the efficacy and safety of this treatment, additional placebo-controlled data are required.
-
Until recently, little was known about the long-term outcome of pulmonary embolism. Long-term mortality and recurrence rates, the case fatality rate of recurrent events, and the frequency of persistent vascular defects remained largely unknown. Improvements in our knowledge of these aspects may help to define the optimal long-term treatment of pulmonary embolism. This review will address these issues. ⋯ Pulmonary embolism has a higher mortality rate than deep venous thrombosis. Patients with pulmonary embolism have no higher risk of recurrence, but any recurrence is more likely to be a new pulmonary embolism than a deep venous thrombosis. A significant number of patients develop persistent perfusion defects after pulmonary embolism.