Abstract：
Acquired von Willebrand syndrome (AVWS) is a rare hematologic disorder characterized by quantitative or qualitative defects of von Willebrand factor (vWF), a protein crucial for normal hemostasis. AVWS has been described in association with several pathologic entities with varied mechanisms. Among these, lymphoproliferative disorders are the most common, with monoclonal gammopathy of undetermined significance (MGUS) being the most frequently reported. AVWS in this setting is commonly associated with the development of bleeding that is clinically challenging to manage due to accelerated clearance of vWF, limiting the utility of many conventional treatment modalities such as DDAVP or vWF/FVIII. We report a case of a 43-year-old male who was sent to our institution for new-onset easy bruising and laboratories concerning for von Willebrand disease (vWD). Further diagnostic workup revealed evidence of an IgG monoclonal gammopathy and findings suggestive of vWF inhibition. Ultimately, he was found to have monoclonal gammopathy of clinical significance (MGCS)-associated AVWS refractory to conventional treatment but responsive to lenalidomide and dexamethasone. This case suggests that lenalidomide may be suitable for patients with AVWS secondary to MGCS.
摘要：
获得性血管性血友病综合征（AVWS）是一种罕见的血液病，其特征是血管性血友病因子（vWF）的定量或定性缺陷，一种对正常止血至关重要的蛋白质。已经描述了AVWS与具有不同机制的几种病理实体相关联。其中,淋巴增生性疾病是最常见的,最常报道的是意义不明的单克隆丙种球蛋白病（MGUS）。这种情况下的AVWS通常与出血的发展有关，由于vWF的加速清除，在临床上具有挑战性。限制了许多常规治疗方式如DDAVP或vWF/FVIII的应用。我们报告了一例43岁的男性，他被送往我们的机构进行新发易发瘀伤和有关vonWillebrand病（vWD）的实验室。进一步的诊断检查显示IgG单克隆丙种球蛋白病的证据和提示vWF抑制的发现。最终,患者被发现患有有临床意义的单克隆丙种球蛋白病(MGCS)相关的AVWS,常规治疗难以治疗,但对来那度胺和地塞米松有反应.该病例表明来那度胺可能适用于MGCS继发的AVWS患者。
