Ouyang H Y, Yu Z J, Yin J, Zhao X J, Wang Z Y, Zhang W, Ma Z N, Su J, Bai X, Ruan C G
Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Collaborative Innovation Center of Hematology, Soochow University, Suzhou 215006, China.
Zhonghua Xue Ye Xue Za Zhi. 2016 Aug 14;37(8):692-5. doi: 10.3760/cma.j.issn.0253-2727.2016.08.012.
To deepen the understanding of acquired von Willebrand syndrome (AVWS).
The clinical data of 3 patients were analyzed and related literature were reviewed.
① Case 1, a 70- year- old male, diagnosed as Waldenstrom macroglobulinemia and AVWS, was presented with spontaneous epitaxis and bruising. The VWF∶Ag level was 16%. Treatment was initiated with VWF concentrates. Two cycles of chemotherapy with Bortezomib, thalidomide and Dexamethasone were followed. Partial remission was achieved. Half- year' follow- up showed no sign of spontaneous hemorrhage. ② Case 2, a 48- year- old female, diagnosed as monoclonal gammopathy of undetermined significance and AVWS, was presented with repeated epitaxis. The VWF∶Ag level was 7%. Because the bleeding was slight and self-relieved, no specific treatment was addressed. She was followed up for one and a half year. ③ Case 3, a 50- year- old man, diagnosed as monoclonal gammopathy of undetermined significance and AVWS, was referred to our hospital for presentation with significant hematomas. VWF∶ Ag was reduced to 12%. VWF- containing cryoprecipitate, plasma, intravenous immunoglobulin and rituximab were used to control his bleeding symptom. During the follow-up, spontaneous hemorrhage still occurred occasionally.
Acquired von Willebrand syndrome presented with heterogeneous symptoms. The level of VWF∶Ag and VWF∶Rco for patients with bleeding disorder should be performed. Abnormal bleeding symptoms in elderly patients without personal or family history of bleeding should prompt consideration of the underlying disorders. Treatment included controlling acute bleeding, curing the underlying diseases and preventing bleeding in high- risk situations. The prognosis of acquired von Willebrand syndrome is mainly related to the underlying diseases.
加深对获得性血管性血友病综合征（AVWS）的认识。
分析3例患者的临床资料并复习相关文献。
①病例1，一名70岁男性，诊断为华氏巨球蛋白血症和AVWS，表现为自发性鼻出血和瘀斑。血管性血友病因子抗原（VWF∶Ag）水平为16%。开始使用VWF浓缩物治疗。随后进行了两个周期的硼替佐米、沙利度胺和地塞米松化疗。达到部分缓解。半年随访显示无自发性出血迹象。②病例2，一名48岁女性，诊断为意义未明的单克隆丙种球蛋白病和AVWS，表现为反复鼻出血。VWF∶Ag水平为7%。由于出血轻微且可自行缓解，未进行特殊治疗。对其进行了一年半的随访。③病例3，一名50岁男性，诊断为意义未明的单克隆丙种球蛋白病和AVWS，因出现大量血肿转诊至我院。VWF∶Ag降至12%。使用含VWF的冷沉淀、血浆、静脉注射免疫球蛋白和利妥昔单抗控制其出血症状。随访期间仍偶尔发生自发性出血。
获得性血管性血友病综合征症状多样。对于有出血障碍的患者应检测VWF∶Ag和血管性血友病因子活性（VWF∶Rco）水平。老年患者无个人或家族出血史但出现异常出血症状时应考虑潜在疾病。治疗包括控制急性出血、治愈基础疾病以及预防高危情况下的出血。获得性血管性血友病综合征的预后主要与基础疾病有关。
