A combined regimen based on bortezomib and glucocorticoids for 6 patients with recurrent/refractory immune thrombotic thrombocytopenic purpura.
10.3760/cma.j.issn.0253-2727.2023.05.010
- VernacularTitle:硼替佐米、糖皮质激素为基础的联合方案治疗6例复发/难治免疫性血栓性血小板减少性紫癜
- Author:
Jie YIN
1
;
Hong TIAN
1
;
Dan Qing KONG
1
;
Yun LI
1
;
Cheng Yuan GU
1
;
De Pei WU
1
;
Zi Qiang YU
1
Author Information
1. Department of Hematology, The First Affiliated Hospital of Soochow University, National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, NHC Key Laboratory of Thrombosis and Hemostasis, Collaborative Innovation Center of Hematology, Soochow University, Suzhou 215006, China.
- Publication Type:Journal Article
- Keywords:
Bortezomib;
Combination therapy;
Thrombotic thrombocytopenic purpura
- MeSH:
Male;
Female;
Humans;
Young Adult;
Adult;
Middle Aged;
Aged;
Bortezomib/therapeutic use*;
Glucocorticoids/therapeutic use*;
Rituximab/therapeutic use*;
Purpura, Thrombotic Thrombocytopenic/drug therapy*;
Purpura, Thrombocytopenic, Idiopathic/drug therapy*;
ADAMTS13 Protein/therapeutic use*
- From:
Chinese Journal of Hematology
2023;44(5):413-417
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To observe the efficacy and adverse reactions of a combination therapy regimen based on bortezomib and glucocorticoids in recurrent/refractory immune thrombocytopenic purpura (iTTP) . Methods: Six patients with recurrent/refractory TTP were included and treated with a glucocorticoid and two courses of bortezomib-based regimen. The clinical remission status of patients, changes in ADAMTS13 activity/ADAMTS13 inhibitor, and the occurrence of treatment-related adverse reactions were observed. Results: Of the 6 patients, 2 were males and 4 were females, with a median age of 21.5 (18-68) years. Refractory TTP was found in 1 case and recurrent TTP in 5 cases. Glucocorticoids were administered with reference to prednisone at 1 mg·kg(-1)·d(-1), and gradually reduced in dosage after achieving clinical remission. Bortezomib is subcutaneously administered at 1.3 mg/m(2) on days 1, 4, 8, and 11 with a 28-day treatment course consisting of 2 courses. Six patients achieved clinical remission after receiving bortezomib as the main treatment. ADMATS13 activity returned to normal in all patients with TTP after treatment, and the ADAMTS13 inhibitor turned negative. Thrombocytopenia is the most common adverse reaction after treatment, with other adverse reactions, including peripheral neuritis and abdominal pain, but ultimately all patients returned to normal. In a median follow-up of 26 (9-41) months, 5 patients maintained sustained remission, and 1 patient relapsed after 16 months of bortezomib treatment. Conclusion: Combination therapy of bortezomib and glucocorticoids has a satisfactory therapeutic effect and controllable adverse reactions for recurrent/refractory iTTP.
