| Objective: Waldenstr(?)m Macroglobulinemia(WM)is a rare type of non-Hodgkin lymphoma with no standard first-line treatment,and the disease is still incurable.Bortezomib alone and in combination with multiple anti-cancer agents has shown substantial activity in WM in both newly diagnosed(ND)and relapsed/refractory(RR)settings.This study evaluated the clinical efficacy,safety,and prognostic factors of bortezomib-based chemotherapy as initial treatment in WM patients,providing clinical evidence for the efficacy and safety of bortezomib.We analyzed the biological characteristics of WM patients at initial diagnosis,and the prognostic factors affecting the efficacy of bortezomib were investigated through follow-up of patients after treatment.Methods: We retrospectively analyzed the clinical data collected from 44 newly diagnosed WM patients treated with bortezomib-based regimens at the Affiliated Hospital of Nantong University from December 2011 to June 2021.The international prognostic stage system of WM(IPSSWM)and the revised-international prognostic stage system of WM(R-IPSSWM)were used for risk stratification.Bortezomib was administered subcutaneously at 1.3 mg/m2 on days 1,4,8,and 11 of the 21-day cycle.After the first course of treatment,patients were given bortezomib weekly for a 35-day cycle in subsequent courses.Efficacy was evaluated using the criteria established at the 7th International Workshop on WM(IWWM).Follow-up was conducted through outpatient and hospitalization records and telephone consultation.Descriptive statistical methods were used to summarize patients’ characteristics and clinical efficacy.Univariate and multivariate analyses were used to assess prognostic factors for overall survival(OS)and progression-free survival(PFS).Results: The median age was 67(49-80)years old from 44 patients,the median number of treatment cycles was 4(2-10),with an overall response rate(ORR)of 93.2%,complete response(CR)rate of 6.8%,and very good partial response(VGPR)rate of 29.5%,partial response(PR)rate of 45.5%.With a median follow-up of 39(13-108)months,the median PFS was 36(5-101)months,while the median OS was not reached.The 2-year overall survival(OS)and 2-year PFS rates were 88.0±6.0% and 59.0±6.0%,respectively.By the last follow-up,eight patients(18.2%)had died.Univariate analysis showed that PFS and OS were shorter in patients with B symptoms(P1=0.014,P2=0.007),the high RIPSSWM(P1=0.035,P2=0.011)and those who did not achieve VGPR(P1=0.032,P2=0.017).Patients with elevated LDH(P=0.038),IPSSWM stage III(P=0.023)also had shorter PFS than those with normal LDH and ISSWM stage I+II.Multivariate analysis showed that B symptom(P=0.023;RR,8.73;95% CI 1.35?56.2)and ≤VGPR(P=0.030;RR,5.79;95% CI 1.19?28.33)were independent adverse prognostic factors of OS.Multivariate analysis also confirmed that IPSSWM stage III(P=0.033;RR,3.22;95%CI 1.10?9.47)and ≤VGPR(P=0.032;RR,2.19;95% CI 1.08?4.47)were associated with poorer PFS.Failure to achieve VGPR was an independent adverse prognostic factor for OS and PFS.Treatment-related peripheral neuropathy(PN)occurred in 4 patients(9.1%).One of them discontinued the subsequent treatment.Six patients(13.6%)developed herpes zoster from taking antiviral drugs irregularly.The dose of dexamethasone was reduced in 2 patients because of hyperglycemia.No patient had “Ig M flare.”Conclusions: The bortezomib-based regimen has shown high efficacy in newly treated WM patients.Weekly subcutaneous administration of bortezomib improved drug tolerance.However,combinations of drugs with different mechanisms are recommended for patients with a high tumor burden.In addition,deep remission can improve patients’ survival. |
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