Page 220 - Haematologica May 2022

Case Reports
with lenalidomide, there was no statistically significant difference in OS between TP53-mutant and wild-type patients.14 Additionally, for CLL patients who have high risk factors including TP53 mutation, the application of lenalidomide maintenance therapy can bring significant clinical benefits.15
Lenalidomide can enhance antibody-dependent cellu- lar cytotoxicity (ADCC), and antibody-dependent cell- mediated phagocytosis (ADCP) of rituximab, showing rational combination strategy with rituximab. Several studies have shown efficacy and safety of the combina- tion of BTKi (ibrutinib or zanubrutinib) with R2 (lenalidomide and rituximab) and with or without chemotherapy (ibrutinib+R2+CHOP, clinicaltrials gov. Identifier: NCT02077166; ibrutinib+R2, clinicaltrials gov. Identifier: NCT02460276; ZR2+CHOP, clinicaltrials gov. Identifiers: EHA2021 EP548), to treat DLBCL and MCL. In a phase II trial (clinicaltrials gov. Identifier: NCT04460248) previously untreated elderly patients with DLBCL will be treated with ZR2 regimen.
Taken together, lenalidomide showed anti-tumor potential for MYC rearrangement-positive DLBCL and TP53-mutant CLL, in addition, ibrutinib+R2 showed effi- cacy for MCL, which may have similar disease character- istics as B-PLL. This patient has both TP53 and MYC mutations along with MYC rearrangement, which pre- dicted the poor outcome with short survival period by conventional chemoimmunotherapy. Therefore, we employed ZR2 as a first line therapy for this patient. As far as we know, this is the first case report to document a successful treatment outcome with ZR2 as upfront therapy for a B-PLL patient.
Although effective standard treatment strategies have not yet been established for patients with B-PLL, we here demonstrate that ZR2 regimen induces a deep and durable response in one B-PLL patient with TP53 and MYC mutations along with MYC rearrangement. Given the poor prognosis of B-PLL and lack of effective estab- lished treatment modalities, this case report could repre- sent a promising indication of ZR2 for B-PLL treatment. Further investigations in large cohort will be needed to characterize the efficacy, safety, and tolerability of this combination treatment.
Lijie Xing, Qiang He, Linna Xie, Hui Wang and Zengjun Li
Department of Hematology, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical Sciences, Jinan, China
Correspondence:
ZENGJUN LI - zengjunli@163.com doi:10.3324/haematol.2021.280259 Received: October 26, 2021.
Accepted: December 16, 2021. Pre-published: December 23, 2021. Disclosures: no conflicts of interest to disclose.
Contributions: LX and ZL designed the study, performed treat- ments, collected and analyzed data, and wrote the manuscript; QH, LX and HW collected data on clinical follow-up. All authors approved the final version of the manuscript.
Acknowledgements: we are grateful to Teru Hideshima and Kenneth Wen at the Dana-Farber Cancer Institute for expert assis- tance in the revision and editing of the manuscript.
Funding: this investigation was supported by the grant ZR2021MH072 to LX from Shandong Provincial Natural Science Foundation, China.
Ethics approval and consent to participate: this study was approved by the Medical Ethical Committee of Shandong Cancer Hospital and Institute. All patients’ samples were obtained with informed consent in accordance with the Declaration of Helsinki.
Data sharing statement: the data sets used and/or analyzed dur- ing the current study are available from the corresponding author on reasonable request.
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