Vol 55, No 3 (2024)
Original research article
Published online: 2024-04-03
Treatment of primary central nervous system lymphoma (PCNSL) – single center experience.
DOI: 10.5603/ahp.99087
Acta Haematol Pol 2024;55(3):151-156.
Abstract
Introduction: Primary central nervous system lymphoma (PCNSL) is a rare extranodal type of non-Hodgkin’s lymphoma. Diffuse large B cell lymphoma (DLBCL) is most often histopathologically confirmed. Modern PCNSL therapy should include an induction and a consolidation phase. Treatment regimens are based on high doses of methotrexate, and the intensity of therapy is adapted to the age of patients and their biological condition.
Material and methods: The aim of this study was a retrospective analysis of the effectiveness and toxicity of therapy used in patients diagnosed with DLBCL of the central nervous system (CNS), treated at the Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation in Wroclaw, Poland, between 2015 and 2022. The analyzed population included 46 patients with a median age of 64.5 years (range: 27–80). Patients were treated according to the R-MATRix, R-MPV, or R-HD-MTX-ARA-C regimen.
Results: After the first-line treatment, complete remission (CR) was achieved in 11 patients, partial remission (PR) in 15, and seven did not respond to the therapy. 11 patients died and two were not qualified for chemotherapy due to their poor general condition. The effectiveness of R-MATRix and R-MPV was similar.
Conclusions: We have shown that the use of the MATRix regimen is associated with greater toxicity and prolonged neutropenia.
Keywords: primary central nervous system lymphomadiffuse large B-cell lymphoma of the central nervous systemimmunochemotherapytoxicity
References
- Alaggio R, Amador C, Anagnostopoulos I, et al. International Agency for Research on Cancer/World Health Organization. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia. 2022; 36(7): 1720–1748.
- Phillips EH, Fox CP, Cwynarski K. Primary CNS lymphoma. Curr Hematol Malig Rep. 2014; 9(3): 243–253.
- Carrabba MG, Reni M, Foppoli M, et al. Treatment approaches for primary CNS lymphomas. Expert Opin Pharmacother. 2010; 11(8): 1263–1276.
- Schaff LR, Grommes C. Primary central nervous system lymphoma. Blood. 2022; 140(9): 971–979.
- Chiavazza C, Pellerino A, Ferrio F, et al. Primary CNS lymphomas: challenges in diagnosis and monitoring. Biomed Res Int. 2018; 2018: 3606970.
- Abrey L, Ben-Porat L, Panageas K, et al. Primary central nervous system lymphoma: the Memorial Sloan-Kettering Cancer Center prognostic model. J Clin Oncol. 2006; 24(36): 5711–5715.
- Omuro A, Correa DD, DeAngelis LM, et al. R-MPV followed by high-dose chemotherapy with TBC and autologous stem-cell transplant for newly diagnosed primary CNS lymphoma. Blood. 2015; 125(9): 1403–1410.
- Ferreri AJM, Cwynarski K, Pulczynski E, et al. International Extranodal Lymphoma Study Group (IELSG). Chemoimmunotherapy with methotrexate, cytarabine, thiotepa, and rituximab (MATRix regimen) in patients with primary CNS lymphoma: results of the first randomisation of the International Extranodal Lymphoma Study Group-32 (IELSG32) phase 2 trial. Lancet Haematol. 2016; 3(5): e217–e227.
- Ferreri AJM, Abrey LE, Blay JY, et al. Summary statement on primary central nervous system lymphomas from the Eighth International Conference on Malignant Lymphoma, Lugano, Switzerland, June 12 to 15, 2002. J Clin Oncol. 2003; 21(12): 2407–2414.
- Mead GM, Bleehen NM, Gregor A, et al. A medical research council randomized trial in patients with primary cerebral non-Hodgkin lymphoma: cerebral radiotherapy with and without cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. Cancer. 2000; 89(6): 1359–1370.
- Glass J, Shustik C, Hochberg FH, et al. Therapy of primary central nervous system lymphoma with pre-irradiation methotrexate, cyclophosphamide, doxorubicin, vincristine, and dexamethasone (MCHOD). J Neurooncol. 1996; 30(3): 257–265.
- Ferreri A, Reni M, Foppoli M, et al. High-dose cytarabine plus high-dose methotrexate versus high-dose methotrexate alone in patients with primary CNS lymphoma: a randomised phase 2 trial. Lancet. 2009; 374(9700): 1512–1520.
- Houillier C, Taillandier L, Lamy T, et al. Whole brain radiotherapy (WBRT) versus intensive chemotherapy with haematopoietic stem cell rescue (IC + HCR) for primary central nervous system lymphoma (PCNSL) in young patients: an intergroup Anocef-Goelams randomized phase II trial (PRECIS). Blood. 2016; 128(22): 782–782.
- Kasenda B, Ferreri AJM, Marturano E, et al. First-line treatment and outcome of elderly patients with primary central nervous system lymphoma (PCNSL)--a systematic review and individual patient data meta-analysis. Ann Oncol. 2015; 26(7): 1305–1313.
- Illerhaus G, Marks R, Müller F, et al. High-dose methotrexate combined with procarbazine and CCNU for primary CNS lymphoma in the elderly: results of a prospective pilot and phase II study. Ann Oncol. 2009; 20(2): 319–325.
- Fritsch K, Kasenda B, Hader C, et al. Immunochemotherapy with rituximab, methotrexate, procarbazine, and lomustine for primary CNS lymphoma (PCNSL) in the elderly. Ann Oncol. 2011; 22(9): 2080–2085.
- Fritsch K, Kasenda B, Schorb E, et al. High-dose methotrexate-based immuno-chemotherapy for elderly primary CNS lymphoma patients (PRIMAIN study). Leukemia. 2017; 31(4): 846–852.
- Illerhaus G, Kasenda B, Ihorst G, et al. High-dose chemotherapy with autologous haemopoietic stem cell transplantation for newly diagnosed primary CNS lymphoma: a prospective, single-arm, phase 2 trial. Lancet Haematol. 2016; 3(8): e388–e397.
- Korfel A, Thiel E, Martus P, et al. Randomized phase III study of whole-brain radiotherapy for primary CNS lymphoma. Neurology. 2015; 84(12): 1242–1248.
- Ostrowska B, Domanska-Czyz K, Romejko-Jarosinska J, et al. Safety and efficacy of induction immunochemotherapy with rituximab, methotrexate, ifosfamide, and vincristine (R-MIV) in patients with primary CNS lymphoma including recent COVID-19 pandemic experience. Br J Haematol. 2023; 201(4): 663–672.
- Mappa S, Marturano E, Licata G, et al. Salvage chemoimmunotherapy with rituximab, ifosfamide and etoposide (R-IE regimen) in patients with primary CNS lymphoma relapsed or refractory to high-dose methotrexate-based chemotherapy. Hematol Oncol. 2013; 31(3): 143–150.
- Naidoo J, Panday H, Jackson S, et al. Optimizing the delivery of antineoplastic therapies to the central nervous system. Oncology (Williston Park). 2016; 30(11): 953–962.
- Makino K, Nakamura H, Hide TI, et al. Salvage treatment with temozolomide in refractory or relapsed primary central nervous system lymphoma and assessment of the MGMT status. J Neurooncol. 2012; 106(1): 155–160.
- Ghesquieres H, Chevrier M, Laadhari M, et al. Lenalidomide in combination with intravenous rituximab (REVRI) in relapsed/refractory primary CNS lymphoma or primary intraocular lymphoma: a multicenter prospective 'proof of concept' phase II study of the French Oculo-Cerebral lymphoma (LOC) Network and the Lymphoma Study Association (LYSA)†. Ann Oncol. 2019; 30(4): 621–628.
- Tun HW, Johnston PB, DeAngelis LM, et al. Phase 1 study of pomalidomide and dexamethasone for relapsed/refractory primary CNS or vitreoretinal lymphoma. Blood. 2018; 132(21): 2240–2248.
- Grommes C, Pastore A, Palaskas N, et al. Ibrutinib unmasks critical role of bruton tyrosine kinase in primary CNS lymphoma. Cancer Discov. 2017; 7(9): 1018–1029.
- Soussain C, Malaise D, Choquet S, et al. Ibrutinib monotherapy for relapse or refractory primary CNS lymphoma and primary vitreoretinal lymphoma: Final analysis of the phase II 'proof-of-concept' iLOC study by the Lymphoma study association (LYSA) and the French oculo-cerebral lymphoma (LOC) network. Eur J Cancer. 2019; 117: 121–130.
- Frigault MJ, Dietrich J, Martinez-Lage M, et al. Tisagenlecleucel CAR T-cell therapy in secondary CNS lymphoma. Blood. 2019; 134(11): 860–866.
