摘要
目的加深对获得性血管性血友病（AVWS）的认识。方法对3例AVWS患者的临床资料进行分析，并复习相关文献。结果①例1，男，70岁，诊断：华氏巨球蛋白血症、AVWS。表现为自发鼻腔、皮肤出血，血管性血友病因子抗原（VWF：Ag）16％。予输注冷沉淀、VDT方案（硼替佐米＋沙利度胺＋地塞米松）化疗2个疗程，获部分缓解，随访半年无活动性出血。②例2，女，48岁，诊断：意义未明的单克隆丙种球蛋白m症、AVWS。表现为反复齿龈出血，VwF：Ag7％，给予观察随诊。③例3，男，50岁，诊断：意义未明的单克隆丙种球蛋白血症、AVWS。表现为轻微外伤后肌肉血肿，VWF：Ag12％，给予血浆、冷沉淀、大剂量静脉丙种球蛋白输注及利妥昔单抗治疗，随访半年中仍有反复出血。结论AVWS临床表现具有异质性；对原因不明的出血患者应进行血浆VWF：Ag检测；成年AVWS患者应注意寻找原发病；治疗包括控制出血、原发病的治疗和预防出血；预后与原发病有关。
Objective To deepen the understanding of acquired von Willebrand syndrome （AVWS）. Methods The clinical data of 3 patients were analyzed and related literature were reviewed. Results 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. 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. Conclusions 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.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2016年第8期692-695,共4页
Chinese Journal of Hematology
