Staal-Viliare A, Didion J, Rio Y
Laboratoire d'hématologie, CHR Metz-Thionville, Groupement des hôpitaux de Metz.
Ann Biol Clin (Paris). 2006 May-Jun;64(3):299-302.
We report a case of acquired von Willebrand syndrome in association with multiple myeloma, diagnosed in a 54 year-old man suffering from sudden onset of mucocutaneous bleeding. The acquired von Willebrand syndrome is a rare bleeding disorder with laboratory findings similar to those of inherited von Willebrand disease. Diagnosis, etiology and pathophysiology of acquired von Willebrand syndrome are reviewed to establish a differential diagnosis with inherited von Willebrand disease. Identification of the underlying disease responsible for the acquired von Willebrand factor defect and the bleeding diathesis is necessary to choose among the therapeutic options the appropriate treatment.
我们报告一例与多发性骨髓瘤相关的获得性血管性血友病综合征，该病例发生在一名54岁突发皮肤黏膜出血的男性患者身上。获得性血管性血友病综合征是一种罕见的出血性疾病，其实验室检查结果与遗传性血管性血友病相似。本文对获得性血管性血友病综合征的诊断、病因及病理生理学进行综述，以与遗传性血管性血友病建立鉴别诊断。识别导致获得性血管性血友病因子缺陷和出血素质的潜在疾病，对于在治疗方案中选择合适的治疗方法是必要的。
