Immunochemotherapy combined with novel agents for Richter syndrome:report of 3 cases  

作　　者：Lijie Xing Hui Wang Dan Liu Qiang He Zengjun Li 

机构地区：[1]Department of Lymphoma,Shandong Cancer Hospital and Institute,Shandong First Medical University and Shandong Academy of Medical Sciences,Jinan,China

出　　处：《Holistic Integrative Oncology》2023年第1期385-389,共5页整合肿瘤学（英文）

基　　金：funded by the National Natural Science Foundation of China(82200224 to LX);the Natural Science Foundation of Shandong Province(ZR2021MH072 to LX).

摘　　要：Objective Richter syndrome(RS)occurs in approximately 2–10%of chronic lymphocytic leukemia(CLL)patients,more often during the disease course than at diagnosis,with a diffuse large B-cell Lymphoma(DLBCL)histology in 95%of cases.Despite great advances in the treatment of CLL in recent years,RS also develops in patients treated with novel agents,as summarized in our case report and review.Methods We summarized 3 patients with RS treated with immunochemotherapy combined with BTK inhibitor(BTKi)or BCL2 inhibitor(BCL2i)and reviewed the literature.Results Three RS patients were summarized.Patient 1 was transformed into DLBCL during dose reductions in ibrutinib and achieved bone marrow(BM)minimal residual disease(MRD)-negative complete response(CR)after rituximab etoposide,dexamethasone,doxorubicin,cyclophosphamide,and vincristine(R-EDOCH)combined with BTKi treatment and sustained progression-free survival(PFS)for more than 2 years.Patient 2,who transformed at the time of diagnosis,progressed after being treated with rituximab,cyclophosphamide,doxorubicin,vincristine,and prednisone(R-CHOP),followed by PD1 antibody combined with cytosine,arabinoside,cisplatin and dexamethasone(DHAP)treatment and PD1 antibody combined with ifosfamide,carboplatin,and etoposide(ICE)treatment.Patient 2 achieved CR after treatment with rituximab,gemcitabine,and oxaliplatin(R-GemOx)combined with BTKi.Patient 3,who transformed at the time of diagnosis with CARD11,TP53,and ATM mutations,progressed after being treated with R-EDOCH combined with BTKi and achieved MRD-negative CR after treatment with R-GemOx and venetoclax,which has continued for 3 months.We summarized new protocols utilizing targeted therapy,such as BTKi acalabrutinib,and checkpoint inhibition,and the potential role of precision medicine in future trials of RS treatment.The efficacy of these protocols as single agents or in combination with immunochemotherapy is currently being evaluated.Conclusion In our study,immunochemotherapy combined with BTKi or BCL2i achieved favorable eff

关 键 词：Richter syndrome DLBCL BTK inhibitor BCL2 inhibitor IMMUNOTHERAPY 

分 类 号：R730.53[医药卫生—肿瘤]