背景：50岁以上的患者多见单克隆丙种病。患者通常无症状。然而,一些患者出现继发性临床表现，现在将其归入实体“临床意义的单克隆γ病”（MGCS）。
方法：这里，我们报告了两例罕见的MGCS病例:获得性血管性血友病综合征(AvWS)和获得性血管性水肿(AAE).
结论：在50岁以上的患者中发现vonWillebrand活动（vWF:RCo）或血管性水肿减少，在没有家族史的情况下,应该提示搜索血液病，特别是，单克隆丙种球蛋白病.
BACKGROUND: Monoclonal gammopathies are common over the age of 50. Patients are usually asymptomatic. However, some patients present with secondary clinical manifestations, which are now grouped under the entity « Monoclonal Gammopathy of Clinical Significance » (MGCS).
METHODS: Here, we report two rare cases of MGCS: an acquired von Willebrand syndrome (AvWS) and an acquired angioedema (AAE).
CONCLUSIONS: The discovery of a decrease in von Willebrand activity (vWF:RCo) or angioedema in a patient over 50 years of age, in the absence of a family history, should prompt a search for a hemopathy and in particular, a monoclonal gammopathy.
