获得性血管性血友病三例报告并文献复习

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...

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Published in中华血液学杂志 Vol. 37; no. 8; pp. 692 - 695
Main Author 欧阳皖雁 余自强 殷杰 赵小娟 王兆钺 张威 马珍妮 苏健 白霞 阮长耿
Format Journal Article
LanguageChinese
Published 南京医科大学附属无锡市第二人民医院血液内科,214002%215006 苏州大学附属第一医院 2016
江苏省血液研究所
卫生部血栓与止血重点实验室,苏州大学血液学协同创新中心
Subjects
Waldenstrom巨球蛋白血症
意义未明单克隆丙种球蛋白病
获得性
血管性血友病
意义未明单克隆丙种球蛋白病
von Willebrand diseases,acquired
血管性血友病,获得性
Waldenstrom巨球蛋白血症
Waldenstrom macroglobulinemia
Monoclonal gammopathy of undetermined significance
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Summary: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。
Bibliography: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 presentati
ISSN:0253-2727
DOI:10.3760/cma.j.issn.0253-2727.2016.08.012