Response and survival for primary therapy combination regimens and maintenance rituximab in Waldenström macroglobulinaemia

Abstract

Summary Waldenström macroglobulinaemia (WM) is a rare and incurable lymphoma. Comparative studies evaluating the efficacy of primary therapy in symptomatic WM patients have not been performed. In this study, we compared response and survival outcomes in WM patients who received primary therapy with cyclophosphamide‐dexamethasone‐rituximab (CDR), bortezomib‐dexamethasone‐rituximab (BDR) and bendamustine‐rituximab (Benda‐R), as well as maintenance rituximab following primary therapy. Analyses were adjusted for relevant clinical factors associated with response and survival. Maintenance rituximab was analysed as a time‐varying covariate. Our study included 182 patients, of which 57 (31%) received Benda‐R, 87 (48%) BDR and 38 (21%) CDR; 116 (64%) received maintenance rituximab. The median time to best response was shorter for Benda‐R and BDR than CDR (18, 20 and 30 months, respectively). Benda‐R and BDR were associated with better median progression‐free survival (PFS) than CDR (5·5, 5·8 and 4·8 years, respectively), and better 10‐year overall survival rates (OS; 95%, 96% and 81%, respectively). Maintenance rituximab was associated with higher rates of major response (97% vs. 68%), and better median PFS (6·8 years vs. 2·8 years) and 10‐year OS rate (84% vs. 66%) when compared to not receiving maintenance. Benda‐R, BDR and maintenance rituximab associate with higher response rates and longer survival in WM patients than CDR and no maintenance, respectively.

Onsets of progression and second treatment determine survival of patients with symptomatic Waldenström macroglobulinemia

“…Since time of onset of progression was significantly associated with SAFTI, theoretically, any treatment aimed at delaying onset of progression, such as maintenance therapy, may improve SAFTI. 37 This assumption remains to be prospectively validated. Finally, tests aimed at identifying progression early (eg, with the monitoring of MYD88(L265P)-positive cells) will likely provide useful prognostic information.…”

Successful treatment of non-IgM lymphoplasmacytic lymphoma by bortezomib-containing regimen: case reports and review of literature

“…A recent report from the Bing Center for WM retrospective analysis in the US has shown a similar response and long-term survival with Bor- or Benda-containing regimens, although the toxicity profile was different. Bor-containing regimen could induce neuropathy, while Benda-regimen could be associated with a risk of secondary leukemia; therefore, Bor-containing regimen is preferred in relatively younger patients [16].…”

Rare lymphomas in routine practice—Treatment and outcome in Waldenström's macroglobulinaemia in the prospective German Tumour Registry Lymphatic Neoplasms

“…Among the existing retrospective, mainly population-based studies on treatment and outcome patterns of patients with WM, 9,24,26,27,[40][41][42][43][44] one large chart-based study also included patients from German clinical practice. 9 In this study, R-CHOP and rituximabbendamustine were the most frequently used chemoimmunotherapy regimens in first-line which is similar to our findings.…”

“…The PFS data presented here (2-year PFS of 87%; 3-year PFS of 83%) are in line with recent retrospective data in patients with WM after first-line treatment with rituximab-bendamustine revealing a 2-year PFS of 88% 40 and a 3-year PFS of approximately 85%. 27 In the phase III RCT on bendamustine plus rituximab vs R-CHOP, those patients with WM who received bendamustine plus rituximab in firstline (n = 22) had a 3-year PFS of about 80%. 14 The latter finding is even more surprising, since patients treated in routine practice usually markedly differ from those selected for clinical trials 30,45 and results suggest that patient outcomes are worse outside a clinical trial setting.…”

“…Although overall survival (OS), WM-related and non-WM-related mortality have improved over time, 26 WM-like other indolent lymphomas-remains incurable. 27 Owing to the scarce evidence from prospective phase III RCTs, routine data are of great importance to identify treatment and survival of patients with WM, particularly, when data have been prospectively collected. In this article, we present data on 139 patients with WM who all underwent systemic treatment.…”