Novel IVS6‐13C>T mutation recognized as a cause of discrepancy between phenotyping and genotyping in KEL*3,4 polymorphism identification

Abstract

tration. It can be proposed that a relapsing TTP known as rituximab-responsive should be retreated in emergency. The rapidity of the rituximab effect is supported by its ability to clear from the blood within a few hours a significant number of B cells, which is correlated to B-cell depletion within marrow and lymph nodes. 3 From undetectable ADAMTS13 at her third episode, a progressive increase was observed under rituximab. Despite the reported correlation between a persistent deficiency in ADAMTS13 and the risk of relapse, this is still a subject of controversy. 4 Thus, the absence of relapse under rituximab maintenance could be explained or not by this. Although plasma levels of rituximab are low or undetectable 4 months after its infusion, a 6-month frequency administration could be enough to achieve the long-term B-cell depletion necessary to maintain the remission. 5 Safety issues concerning a prolonged administration of rituximab should be outlined, but few data exist. 1 Early front-line therapy with rituximab for relapsing TTP patients previously responsive to the same drug could be a therapeutic option. For those who achieve remission, a rituximab maintenance schedule could also be considered, ideally within clinical trials, because our experience with this one patient is not enough to be raised as a solid recommendation.