...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>Clinical Laboratory >Rituximab Failure In A Patient With Monoclonal Gammopathy Of Undetermined Significance (mgus)-associated Acquired Von Willebrand Syndrome
【24h】

Rituximab Failure In A Patient With Monoclonal Gammopathy Of Undetermined Significance (mgus)-associated Acquired Von Willebrand Syndrome

机译：不确定意义（mgus）相关性获得性冯·威兰布兰德综合征的单克隆丙种球蛋白病患者的利妥昔单抗治疗失败

获取原文
获取原文并翻译 | 示例
           
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Acquired von Willebrand syndrome (aVWS) is a rare coagulation disorder with an estimated prevalence in the general population of about 0.04% to 0.13%. Underlying conditions include myeloproliferative diseases, hypothyro-idism, cardiovascular diseases, malignant tumors, treatment with drugs such as valproate or ciprofloxacin, and, in more than one third of patients, lymphoproliferative disorders - particularly MGUS.rnThe clinical course of a patient with an aVWS was briefly described. The patient was diagnosed as having aVWS associated with a MGUS on the basis of a characteristic coagulation profile: prolonged activated partial thrombo-plastin time, very long closure times in platelet function analysis (collagen-epinephrine and collagen-ADP cartridges); factor VIII activity, VWF ristocetin cofactor activity, VW collagen binding activity and VWF antigen levels were low, and VW propeptide level was normal. IgG autoantibodies against VWF were detected using an ELISA; serum immunoelectrophoresis identified a monoclonal IgG k protein at concentration of about 2 g/I. At the time of diagnosis, the aVWS was not associated with overt bleeding complications and the patient was fol-lowed-up without specific treatment.
机译：获得性von Willebrand综合征（aVWS）是一种罕见的凝血障碍，估计在一般人群中的患病率约为0.04％至0.13％。基础疾病包括骨髓增生性疾病，甲状腺功能减退症，心血管疾病，恶性肿瘤，用丙戊酸盐或环丙沙星等药物治疗，以及三分之一以上的患者发生淋巴增生性疾病-特别是MGUS。简要描述。根据特征性凝血特征，该患者被诊断为患有MGUS相关的avWS：延长的活化部分凝血活酶时间，血小板功能分析中的封闭时间非常长（胶原蛋白-肾上腺素和胶原蛋白-ADP柱）； VIII因子活性，VWF瑞斯托菌素辅助因子活性，VW胶原蛋白结合活性和VWF抗原水平低，而VW前肽水平正常。使用ELISA检测针对VWF的IgG自身抗体；血清免疫电泳鉴定了浓度约为2 g / l的单克隆IgG k蛋白。在诊断时，aVWS与明显的出血并发症无关，患者未经特殊治疗就被随访。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号