Venetoclax combined with hypomethylating agents in treatment of blastic plasmacytoid dendritic cell neoplasm: report of 5 cases and review of literature
10.3760/cma.j.cn115356-20231117-00078
- VernacularTitle:维奈克拉联合去甲基化药物治疗母细胞性浆细胞样树突细胞肿瘤5例并文献复习
- Author:
Mao JIN
1
;
Qian WANG
;
Feng CHEN
;
Xuefeng HE
;
Xiaohui HU
;
Suning CHEN
;
Xiao MA
;
Haiwen HUANG
Author Information
1. 苏州大学附属第一医院血液科，苏州 215006
- Keywords:
Hematologic neoplasms;
Venetoclax;
Azacitidine;
Decitabine
- From:
Journal of Leukemia & Lymphoma
2024;33(6):343-348
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the efficacy and safety of venetoclax (VEN) combined with hypomethylating agents (HMA) in the treatment of blastic plasmacytoid dendritic cell neoplasms (BPDCN).Methods:A retrospective case series study was conducted. The clinical data of 5 patients with BPDCN treated with VEN combined with azacitidine (AZA) or decitabine (DAC) in the First Affiliated Hospital of Soochow University and Suzhou Hongci Blood Disease Hospital from February 2017 to July 2023 were collected, and the therapeutic effect, adverse reaction and prognosis of all 5 patients were summarized.Results:All 5 BPDCN patients were male with the median onset age [ M ( Q1, Q3)] of 66 years (51 years, 73 years), of which 4 cases were presented with skin lesions and 1 case was presented with lymphadenopathy as the primary symptom. As for the treatment, 3 patients were initially treated with VEN in combination with AZA induction regimen, among which 2 patients achieved complete remission with incomplete blood count recovery (CRi) after 2 cycles of treatment, survived for 26.5 months and 14.6 months, respectively and finally died, and 1 patient achieved partial remission after 1 cycle of treatment and he still survived after 3-month follow-up; 1 patient was initially treated with VEN in combination with DAC induction regimen, and achieved clinical complete remission of non-active disease with residual skin abnormalities after 2 cycles of treatment followed by allogeneic hematologic stem cell transplantation (allo-HSCT) and he was in the state of disease-free survival for 15-month; and another 1 patient experienced a relapse after treatment with acute lymphocytic leukemia-like regimen in combination with allo-HSCT and again achieved CRi after 2 treatment courses of VEN in combination with AZA regimen, and he was in the state of disease-free survival for 30-month follow-up. Treatment-related haematological adverse effects of VEN combined with HMA were mainly neutropenia with fever, reduction of hemoglobin and thrombocytopenia; and non-haematological adverse effects were mainly gastrointestinal reactions such as nausea and vomiting. These adverse events improved with symptomatic supportive therapy, and no treatment-related deaths occurred. Conclusions:BPDCN patients who are unable to tolerate intensive chemotherapy regimens at initial time of diagnosis may attempt induction therapy with VEN+HMA regimen, which has a manageable adverse reaction and may serve as a bridge to allo-HSCT.
