血液疾患と輸血ジャーナル

概要

Efficiency of Intensive Immune -Suppression Treatment with Rituximab in Acquired Thrombotic Thrombocytopenic Purpura: A Chinese Case study

Fang Hu, Ziqiang Yu, Jian Su, Ling Sun, Tianqin Wu, Zhaoyue Wang, Xia Bai and Changgeng Ruan

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia (MAHA) and functional failure of the organs due to ADAMTS13 deficiency. ADAMTS13 inhibitor is the main contributor of pathogenesis in acquired TTP. With the help of retrospective analysis the researchers tried to evaluate the effectiveness of rituximab in preventing the acquired TTP in China. Out of 27 patients with acquired TTP that have registered from 2006 to 2015, eleven cases started rituximab infusion before remission, while sixteen others began rituximab infusion after remission. Twenty-three cases got rituximab with standard doses (375 mg/m2 , weekly for 4 weeks) and four patients were administered reduced doses (100mg, weekly for 4 weeks) after remission. Till date, there was no patient reported with the acquired TTP relapsed during the follow-up sessions, whenever rituximab treatment was initiated (before or after remission), or what dose of rituximab was administrated (standard dose or reduced dose). There were mild controllable side-effects reported in the case of four patients. The study concludes that rituximab can act remarkably in relieving acquired TTP, while preventing the relapse.