Bleeding complications after arthroscopy in a JAK2V617F-positive patient with essential thrombocythemia and acquired von Willebrand syndrome (AVWS)

Int J Hematol (2015) 101:405410

CASE REPORT

DOI 10.1007/s12185-014-1707-7

Bleeding complications after arthroscopy in a JAK2V617Fpositive patient with essential thrombocythemia and acquired von Willebrand syndrome (AVWS)

Joanna RupaMatysek Krzysztof Lewandowski Maria Lewandowska Ewelina Wojtasiska Marzena Liliana Wojtaszewska Micha Walczak Ksenia Bykowska Mieczysaw Komarnicki

Received: 14 February 2014 / Revised: 17 November 2014 / Accepted: 19 November 2014 / Published online: 29 November 2014 The Japanese Society of Hematology 2014

Keywords Essential thrombocythemia Bleeding complications Acquired von Willebrand syndrome Arthroscopy

Introduction

Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder characterised by structural or functional defects of von Willebrand factor (VWF), most frequently associated with an underlying disorder such as a neoplasm (68 %) or autoimmune disorder 2 %, according to the Registry of the International Society on Thrombosis and Hemostasis (ISTH) [1]. Several pathogenic mechanisms responsible for the structural or functional disturbances of VWF in AVWS include: (1) both specic autoantibodies and nonspecic autoantibodies that form circulating immune complexes and enhance the clearance of VWF, (2) adsorption of VWF onto malignant cell or other cell surfaces, (3) enhanced shear stress, (4) decreased synthesis and (5) increased proteolytic degradation of VWF by specic or nonspecic proteases. Adsorption of VWF to platelets and increased proteolytic degradation of VWF are the main pathogenic mechanisms of AVWS in myeloproliferative neoplasms (MPN), especially in essential thrombocythemia (ET) [2]. The clinical manifestations of AVWS in patients with ET depend on platelet count and function, VWF plasma content and its multimeric pattern. By contrast, a consumption of high-molecular weight VWF mul-timers (HMW-VWF), due to spontaneous chronic platelet aggregation in vivo and/or increasing VWF clearance from plasma, appear to be the most common mechanisms leading to haemorrhagic events in patients with ET [1].These events are frequently observed if the platelet count is extremely high and exceeds 1000 109/L. This report

Abstract Acquired von Willebrand syndrome (AVWS) is an acquired bleeding disorder with clinical and laboratory features similar to those of the inherited form of the disease. AVWS is reported in many disorders, most frequently in myeloproliferative neoplasms and in, among others, essential thrombocythemia (ET). Interestingly, ET is associated with both the thrombotic and haemorrhagic complications, which occur in 20 % and 530 % of patients, respectively. The present report concerns a 38-year-old...