Rupa-Matysek Joanna, Lewandowski Krzysztof, Lewandowska Maria, Wojtasińska Ewelina, Wojtaszewska Marzena Liliana, Walczak Michał, Bykowska Ksenia, Komarnicki Mieczysław
Department of Haematology, Poznan University of Medical Sciences, Szamarzewskiego 84, 60-569, Poznan, Poland,
Int J Hematol. 2015 Apr;101(4):405-10. doi: 10.1007/s12185-014-1707-7. Epub 2014 Nov 29.
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 5-30 % of patients, respectively. The present report concerns a 38-year-old man, suffering from ET, who presented with two episodes of post-arthroscopic joint bleeding after synovectomy required for the treatment of synovial hypertrophy and chronic left knee joint synovitis. We discuss the current diagnostic approaches, as well as the risk factors predisposing to bleeding and its management, in patients with essential thrombocythemia.
获得性血管性血友病综合征(AVWS)是一种获得性出血性疾病,其临床和实验室特征与遗传性血管性血友病相似。许多疾病都有AVWS的报道,最常见于骨髓增殖性肿瘤,尤其是原发性血小板增多症(ET)。有趣的是,ET与血栓形成和出血并发症相关,分别发生在20%和5%-30%的患者中。本报告涉及一名38岁患有ET的男性,他在因滑膜肥大和慢性左膝关节滑膜炎接受滑膜切除术后,关节镜检查后出现了两次关节出血。我们讨论了原发性血小板增多症患者目前的诊断方法、出血的危险因素及其管理。