Sailler L, Ecoiffier M, Cadroy Y, Couret B, Sié P, Mazurier C, Arlet-Suau E, Pourrat J P
Service de néphrologie, pavillon Sénac, CHU Purpan, Toulouse, France.
Rev Med Interne. 1996;17(11):929-32. doi: 10.1016/0248-8663(96)88123-9.
Acquired von Willebrand's disease associated with a monoclonal gammopathy and angiodysplasia of the gut is a rare disorder. It is sometimes complicated by chronic intestinal bleeding and severe anemia, that is poorly responsive to usual treatments. We report such a new case that has been revealed by anemia, and characterised by the absence of the high-molecular weight multimers. The correction of the hemostasis defect and of anemia were related to the reappearance of the high-molecular weight multimers, that was achieved only after high-dose intravenous immunoglobulin courses. The perfusions were performed every 3 weeks for 2 years without loss of efficiency, that could be explained by the dissociation of immunoglobulin-von Willebrand's factor complex.
获得性血管性血友病综合征与单克隆丙种球蛋白病及肠道血管发育异常相关，是一种罕见的疾病。它有时会并发慢性肠道出血和严重贫血，对常规治疗反应不佳。我们报告了这样一例新病例，该病例因贫血而被发现，其特征是缺乏高分子量多聚体。止血缺陷和贫血的纠正与高分子量多聚体的重现有关，这仅在大剂量静脉注射免疫球蛋白疗程后才得以实现。灌注每3周进行一次，持续2年且效果不减，这可以通过免疫球蛋白 - 血管性血友病因子复合物的解离来解释。
