Abstract
Waldenström's macroglobulinemia (WM) is a B-cell proliferative malignancy characterized by immunoglobulin M monoclonal gammopathy and bone marrow infiltration by lymphoplasmacytic cells. Clinical features and cytogenetics of WM in Asia including Republic of Korea remain unclear. Moreover, no study has reported treatment outcomes in patients with WM treated with novel agent combined with conventional chemotherapy. This study investigated clinical features and assessed treatment outcomes with novel agent and conventional chemotherapy in Republic of Korea. Data from all (n = 71) patients with newly diagnosed WM at 17 hospitals who received chemotherapy between January 2005 and December 2012 were collected retrospectively. The median age of patients was 66 years (range: 37–92 years) and male to female ratio was 5 : 1. Patients treated with novel agent combined chemotherapy displayed higher overall response rate (ORR) compared to conventional chemotherapy alone (92.9% versus 52.6%, P = 0.006). The 5-year overall survival rate was 62.6% (95% confidence interval: 34.73–111.07). Use of novel agents produced higher ORR but survival benefit was not apparent due to the small number of patients and short follow-up duration. Further studies are needed to confirm the efficacy of novel agents in patients with WM.
Highlights
The consensus group at the Second International Workshop on Waldenstrom’s macroglobulinemia (WM) in 2002 redefined WM as a distinct clinicopathologic entity characterized by bone marrow infiltration by lymphoplasmacytic lymphoma (LPL) and immunoglobulin M (IgM) monoclonal gammopathy [1]
The pretreatment evaluation included a physical examination with performance status evaluation, complete blood cell count with differential count, blood chemistry including lactase dehydrogenase (LDH), protein electrophoresis (PEP), IgM, free light chain kappa and lambda, bone marrow biopsy, chromosomal study, fluorescence in situ hybridization (FISH), and computed tomography (CT) of the chest, abdomen, and pelvis
We investigated independent prognostic factors associated with survival in above clinical and laboratory parameters
Summary
The consensus group at the Second International Workshop on Waldenstrom’s macroglobulinemia (WM) in 2002 redefined WM as a distinct clinicopathologic entity characterized by bone marrow infiltration by lymphoplasmacytic lymphoma (LPL) and immunoglobulin M (IgM) monoclonal gammopathy [1]. Diagnostic criteria for WM are IgM monoclonal gammopathy of any concentration, bone marrow infiltration by small lymphocytes showing plasmacytoid or plasma cell differentiation, intertrabecular pattern of bone marrow infiltration, and surface IgM+, CD5±, CD10−, CD19+, CD20+, CD22+, CD23−, CD25+, CD27+, FMC7+, CD103−, and CD138− immunophenotype [2]. The incidence rate of WM is lower in the Republic of Korea than those in the USA, which was documented to be about 0.3 per million personyears according to data of the National Cancer Information Center in the Republic of Korea
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