Prognostic factors and primary treatment for Waldenström macroglobulinemia – a Swedish Lymphoma Registry study

Abstract

We present a nationwide prospective Swedish registry-based study of Waldenström macroglobulinaemia (WM), that focuses on incidence and survival in relation to clinical prognostic factors and primary systemic therapies. A total of 1511 patients with WM and lymphoplasmocytic lymphoma (LPL) were registered in the Swedish Lymphoma Registry (SLR) between 1 January 2000 and 31 December 2014. The age-adjusted incidence of WM/LPL was 11·5 per million persons per year, three times higher than the reported incidence worldwide. Medical records were retrieved for 1135 patients (75%). A retrospective review showed that 981 (86·1%) of these patients fulfilled the World Health Organization diagnostic criteria for WM and these patients were analysed further. The overall survival (OS) improved between two periods - 2000-2006 and 2007-2014 - with a five-year OS of 61% and 70%, respectively. Significant prognostic factors for OS, evaluated at the time of diagnosis, were age, elevated lactate dehydrogenase level and haemoglobin ≤115 g/l for patients receiving therapy 0-3 months after diagnosis, and age, poor performance status, haemoglobin ≤115 g/l, and female sex in "watch and wait" patients (multivariable analysis). The level of the IgM monoclonal immunoglobulin had no significant prognostic value. Rituximab included in first-line therapy was associated with improved survival.

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

“…9 Three-year OS of patients included in our analysis was 87%, a finding that agrees with results from other (retrospective and prospective) studies: in patients with WM who received any systemic first-line treatment within the period of 2000 to 2014, 3-year OS ranged from 63% to approximately 95%. 9,11,26,27,41,43 Notably, the lowest 3-year OS has been observed in patients with the 'highest' median age of 78 years 43 and the highest 3-year OS in patients of the 'youngest' median age (65 years). 9 With 72 years in median at start of first-line treatment, the age of our registry cohort is very similar to that of the prospective SLR cohort having a median age of 73 years.…”

“…39,40 Our findings on treatment patterns vary from those of the Swedish Lymphoma Registry (SLR) study on prognostic factors and treatment for WM that-to our knowledge-is the only prospective population-based study published so far. 11 Of 203 patients with documented systemic first-line treatment between 2000 and 2014, 153 were treated between 2007 and 2014 and mainly received cyclophosphamide-(rituximab)-based regimens (28%, of which 6% were R-CHOP-like regimens) and chlorambucil (27%, mostly without rituximab). Only in 7% of patients rituximab-bendamustine was used.…”

“…Only in 7% of patients rituximab-bendamustine was used. 11 These differences might reflect differences in patient cohorts, national guidelines and health care systems between Sweden and…”

“…10 Choice of first-line treatment is guided by the clinical presentation of the disease and/or complications 1 and individual patient's characteristics. 11 Since WM incidence is low, few phase III randomised clinical trials (RCTs) have been conducted so far. 9,10,[12][13][14][15] Thus, treatment recommendations and individual decisions are mostly based on phase II data.…”

Incidence and inheritance of hyperphosphorylated paratarg-7 in patients with Waldenstrom’s macroglobulinaemia in Sweden

“…In Sweden, the incidence of WM/LPL is high; 11.5, measured as million persons per year, with an even higher incidence in the northern counties; Norrbotten County = 17.6; and Vasterbotten County = 14.3 million persons per year [17] and with an aggregation of families in these counties. This high incidence and family aggregation in one region may relate to heredity, but environmental and other unknown factors may also contribute [9].…”