Primary therapy and relative survival in patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia: a population‐based study in the Netherlands, 1989–2018

Abstract

It is unclear how treatment advances impacted the population-level survival of patients with lymphoplasmacytic lymphoma/Waldenstr€ om macroglobulinaemia (LPL/WM). Therefore, we assessed trends in first-line therapy and relative survival (RS) among patients with LPL/WM diagnosed in the Netherlands between 1989 and 2018 (N = 6232; median age, 70 years; 61% males) using data from the nationwide Netherlands Cancer Registry. Patients were grouped into three age groups (<65, 66-75 and >75 years) and four calendar periods. Overall, treatment with anti-neoplastic agents within 1 year post-diagnosis gradually decreased over time, following a broader application of an initial watch-and-wait approach. Approximately 40% of patients received anti-neoplastic therapy during 2011-2018. Furthermore, use of chemotherapy alone decreased over time, following an increased application of chemoimmunotherapy. Detailed data among 1596 patients diagnosed during 2014-2018 revealed that dexamethasonerituximab-cyclophosphamide was the most frequently applied regimen; its use increased from 14% to 39% between 2014 and 2018. The 5-year RS increased significantly over time, particularly since the introduction of rituximab in the early-mid 2000s. The 5-year RS during 1989-1995 was 75%, 65%, and 46% across the age groups compared to 93%, 85%, and 79% during 2011-2018. However, the survival improvement was less pronounced after 2011. Collectively, the impressive survival improvement may be accounted for by broader application of rituximab-containing therapy. The lack of survival improvement in the post-rituximab era warrants studies across multiple lines of therapy to further improve survival in LPL/ WM.

Baseline 18F-FDG PET/CT May Portend the Prognosis of Patients With Waldenström Macroglobulinemia/Lymphoplasmacytic Lymphoma After First-Line Treatment

“…The treatment regimens may depend on patients' age, performance status, comorbidities, genomic profiles, and economic aspects. 5,29 In our cohort, the most commonly used regimen was immunochemotherapy with rituximab (63.6% of the patients), followed by chlorambucil-containing regimens, other combination of chemotherapy, and single-agent rituximab. We documented an 84.8% overall response rate after first-line treatment in our cohort, consistent with the results in previous studies.…”

“…Similarly to other indolent lymphomas, treatment is indicated for WM/LPL only in cases of symptomatic disease, but the treatment landscape of WM/LPL is heterogeneous. The treatment regimens may depend on patients' age, performance status, comorbidities, genomic profiles, and economic aspects 5,29 . In our cohort, the most commonly used regimen was immunochemotherapy with rituximab (63.6% of the patients), followed by chlorambucil-containing regimens, other combination of chemotherapy, and single-agent rituximab.…”

Dutch Physician’s Perspectives on Diagnosis and Treatment of Waldenström’s Macroglobulinemia Before and After the Implementation of a National Guideline

“…This switch in preferred treatment was confirmed in a nationwide Dutch patient registry, showing decrease of R-COP/CVP from 12% to 2% and increased use of DRC from 14% to 39% (between 2014 and 2018). 22 We suspect that this switch is mostly related to the publication of the first Dutch WM guideline, which suggested DRC as a preferred first-line treatment strategy in contrast to international guidelines at that time that suggested a range of first-line therapies without a preferred option. 16 The use of vincristine was discouraged considering the high rates of peripheral neuropathy without clearly added efficacy.…”

Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management

“…Patients under the age of 70 have a median survival in excess of 10 years; those 70–79, approximately 7 years; and those 80 or older, approximately 4 years. In patients with Waldenstrom over age 65 at diagnosis, the most common cause of death is not cancer related 5 . Clonal hematopoiesis is present in 14% of patients with macroglobulinemia.…”

“…In patients with Waldenstrom over age 65 at diagnosis, the most common cause of death is not cancer related. 5 Clonal hematopoiesis is present in 14% of patients with macroglobulinemia. These patients are more likely to progress from IgM MGUS or smoldering macroglobulinemia to symptomatic disease.…”