Combining Low-Dose Rituximab and Bortezomib as Immunosuppressive Therapy for Acquired Hemophilia A: 6 Case Series

作者全名："Fu, Yuhan; Chen, Shu; Wang, Anzi; Luo, Jingyuan; Li, Qing"

作者地址："[Fu, Yuhan; Chen, Shu; Wang, Anzi; Luo, Jingyuan; Li, Qing] Chongqing Med Univ, Dept Hematol, Affiliated Hosp 2, 74 Linjiang Rd, Chongqing 40010, Peoples R China"

通信作者："Chen, S (通讯作者)，Chongqing Med Univ, Dept Hematol, Affiliated Hosp 2, 74 Linjiang Rd, Chongqing 40010, Peoples R China."

来源：INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION

ESI学科分类：CLINICAL MEDICINE

WOS号：WOS:000993892400001

JCR分区：Q4

影响因子：0.9

年份：2024

卷号：40

期号：1

开始页：157

结束页：160

文献类型：Article

关键词：Acquired hemophilia A; Immunosuppression treatment; Bortezomib; Rituximab

摘要："Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against factor VIII. Persistently positive inhibitor titers increase the risk of bleeding. Upfront combined regimen with multiple mechanisms has the potential to shorten remission time to lower bleeding risk and reduce immunosuppressor exposure time. We administered lowered dose of rituximab and bortezomib in combination with corticosteroids and cyclophosphamide (rituximab: 100 mg weekly x 4 or 500 mg once in week 1, bortezomib: 0.65 mg/m2 once in week 1, prednisone: 1 mg/kg daily, cyclophosphamide: 11.5-2 mg/kg daily). We retrospectively analyzed 6 cases (male = 3, female = 3, median age = 51 years) treated on this therapeutic regimen. All patients achieved complete remission (range from19 to 66 days); adverse event: infection (1/6). This is the first time that this regimen has been reported for treating AHA. It has shown good therapeutic efficacy and the potential to shorten the time to CR. The incidence of adverse events is within a reasonable range. This regimen is feasible and offers a new alternative treatment for AHA."

基金机构：　

基金资助正文：