PLG and atypical hemolytic-uremic syndrome: It has been suggested that heterozygous mutations of the PLG gene result in dysfunctional plasminogen with decreased activity (“dysplasminogenemia”), which might be a predisposing factor for thrombotic events11 and atypical hemolytic uremic syndrome.12 Even though patients with dysplasminogenemia have a normal immunoreactive plasminogen level, the functional activity of plasminogen is significantly decreased due to abnormalities in the mutant PLG protein.