New Developments in Diagnosis and Management of Acquired Hemophilia and Acquired von Willebrand Syndrome

Acquired hemophilia A and acquired von Willebrand syndrome are rare, but life-threatening bleeding disorders that require prompt diagnosis and treatment by hematologists. Acquired hemophilia A is defined as an acquired severe bleeding tendency caused by autoantibody formation against coagulation factor VIII. Acquired von Willebrand syndrome is characterized by a new onset bleeding tendency caused by a reduced concentration and/or function of von Willebrand factor. These disorders are associated with a variety of underlying disorders, including various hematological malignancies, for example, plasma cell disorders, lymphoproliferative disorders, monoclonal gammopathy of undetermined significance, and myeloproliferative neoplasms. It is of utmost important to recognize these acquired bleeding disorders in these patients who are at risk for severe bleeding, and to perform additional diagnostic hemostasis laboratory evaluation. This will enable immediate diagnosis of the acquired bleeding disorder and management of both the bleeding episodes and the causative underlying disorder. In recent years, several new etiological factors for acquired hemophilia A, such as treatment with immune checkpoint inhibitors or DPP-4 inhibitors and SARS-CoV2 infection, and for acquired von Willebrand syndrome, for example, left ventricular assist devices, have been identified and also new treatment options have become available. In this concise review, the most recent data on etiology, diagnosis, and treatment of acquired bleeding disorders are presented and discussed

Balans in beweging

Rede, Rede uitgesproken bij de aanvaarding van het ambt van bijzonder hoogleraar met als leeropdracht hemostase en trombose aan het Erasmus MC, faculteit van de Erasmus Universiteit Rotterdam op 5 februari 201

Clinical studies on fibrinolysis inhibitors synthesized by the liver

In this thesis studies on congenital and acquired disorders of the fibrinolytic system, with emphasis on the inhibitors of the fibrinolytic system, are described. This was initiated to obtain more information on the fibrinolytic system, and to obtain insight into the mechanism of enhancement of fibrinolysis in patients with liver disease. In chapter 2, a concise review on the fibrinolytic system, on the role of the liver in the regulation of the fibrinolytic system and an overview on the literature on fibrinolysis in liver disease are given. In the other chapters biochemical and clinical studies on fibrinolysis inhibitors are reported. In the last two chapters we report investigations on the fibrinolytic system in patients with liver cirrhosi

Construction and functional analysis of hybrid interleukin-6 variants Characterization of the role of the C-terminus for species specificity

AbstractWe have constructed several hybrid human interleukin-6 (IL-6) variants in which the carboxyl-terminus, which includes a receptor binding site of IL-6 has been replaced with the C-terminus of various proteins homologous to human IL-6, IL-6 hybrids with the C-terminus of human growth hormone and human granulocyte-colony stimulating factor maintain part of the biological activity of human IL-6. Replacing the C-terminus of human IL-6 with the C-terminus of mouse and rat IL-6 resulted in a normal or increased activity on a mouse cell line; however, this gave a low (to 200-fold less) activity on a human cell line compared to wild-type human IL-6. We therefore conclude that the C-terminus of IL-6 plays in important role in the species specificity of IL-6