1,721,021 research outputs found

    Recombinant factor VIIa. An update on its clinical use

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    Recombinant activated factor VII (rFVIIa, NovoSeven) has been successfully used to treat bleeding episodes in patients with antibodies against coagulation factors VIII and IX. In recent years, rFVIIa has also been employed for the management of uncontrolled bleeding in a number of congenital and acquired haemostatic abnormalities. Based on a literature search, this review examines the current knowledge on therapy with rFVIIa, from the now well-standardized uses to the newer and less well-characterised clinical applications

    High prevalence of sustained remission of idiopathic thrombocytopenic purpura after helicobacter pylori eradication

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    We studied the effect of Helicobacter pylori (H. pylori ) eradication in 43 consecutive H. pylori-infected patients with idiopathic thrombocytopenic purpura. H. pylori was eradicated with antibiotics in 41 of them (95.3%). The difference between the mean platelet count before and after therapy was statistically significant (54.3109/l vs. 119.1109/l; P < 0.001). A sustained remission was observed in 20 patients (48.8%), after a median follow-up of 31.2 months. None of the patients still infected by H. pylori after therapy reached normal platelet values. The long-term follow-up confirms the efficacy of H. pylori eradication in H. pylori-infected ITP patients

    Advances in the pathogenesis, diagnosis and treatment of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome

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    The thrombotic microangiopathies are microvascular occlusive disorders characterized by hemolytic anemia caused by fragmentation of erythrocytes and thrombocytopenia due to increased platelet aggregation and thrombus formation, eventually leading to disturbed microcirculation with reduced organ perfusion. Depending on whether brain or renal lesions prevail, two different entities have been described: thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). However, not rarely the clinical distinctions between these two conditions remain questionable. Recent studies have contributed greatly to our current understanding of the molecular mechanisms leading to TTP and HUS. In this review, we briefly focus on the most important advances in the pathophysiology, diagnosis and treatment of these two thrombotic microangiopathies

    The potential role of recombinant activated FVII in the management of critical oncohematological bleeding: a systematic review.

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    Recombinant activated factor VII (rFVIIa) is an hemostatic agent that was originally developed for the treatment of hemorrhage in patients with hemophilia and inhibitors. However, in the last few years rFVIIa has been employed with success in a broad spectrum of congenital and acquired bleeding conditions. In this systematic review we present the current knowledge on the use of this drug in patients suffering from hemato-oncological disorders, which are quite commonly complicated by severe hemorrhage. On the whole, data in the literature suggest a potential role for rFVIIa in the management of bleeding unresponsive to standard therapy in patients with hematological malignancies, including those undergoing bone marrow transplant. However, the vast majority of the currently available data are derived from uncontrolled studies including single cases or small series of patients. Thus, further trials with larger numbers of patients are needed to establish the most appropriate doses and timing of rFVIIa and to assess its efficacy and safety in this setting

    Analysis of thyroid hormone status in 131 consecutive individuals with low von Willebrand factor levels.

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    Analysis of thyroid hormone status in 131 consecutive individuals with low von Willebrand factor levels

    Rituximab for the treatment of childhood chronic idiopathic thrombocytopenic purpura and hemophilia with inhibitors

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    Rituximab, a monoclonal chimeric antibody to the CD20 antigen, is an effective treatment for non-Hodgkin lymphomas. Moreover, rituximab has also shown efficacy in various autoimmune disorders. In this review, we will focus on the use of rituximab in childhood disorders of hemostasis associated with inhibitor formation. Although the results presented suggest that rituximab can be useful in the treatment of this subset of pediatric patients, most of the data come from isolated case reports or descriptions of small, uncontrolled series. Therefore, large, prospective, and randomized trials are needed to confirm the positive, preliminary results

    Pathogenesis, laboratory and clinical characteristics of Helicobacter pylori-associated immune thrombocytopenic purpura.

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    Idiopathic thrombocytopenic purpura (ITP) is a common autoimmune disease mediated by autoantibodies against platelet glycoproteins. This hemorrhagic disorder may be primary or secondary to various illnesses, including lymphoproliferative, autoimmune, or infectious diseases. Among the latter causes, there is increasing laboratory and clinical evidence that documents a pathogenic role of Helicobacter pylori infection in ITP. The aim of this review is to analyze the current knowledge on the pathogenic, diagnostic, clinical, and therapeutic characteristics of H. pylori-associated ITP
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