The incidence and survival of Waldenström's Macroglobulinaemia in South East England

Abstract

Waldenström's Macroglobulinaemia (WM) is an uncommon B-cell lymphoproliferative disorder defined as a predominately inter-trabecular bone marrow infiltration of small lymphocytes with an IgM monoclonal gammopathy. There are little reliable incidence and survival data for the disease in the UK since epidemiological studies have usually grouped it with other plasma cell dyscrasias. This study uses data from the South Thames Haematology Register and the Thames Cancer Registry for South East England to describe the incidence and survival of WM, and the influence of selected clinical factors on survival. Between 1999 and 2001, there were 152 new cases of WM recorded in the South Thames Haematology Register, giving an age standardised rate of 0.55 per 100,000 European standard population (0.73 for males and 0.42 for females). The incidence increased with age, and the median age at diagnosis was 75 years (range 45-93 years). The estimated 5 year survival was 57% (95% CI: 47-66%). Age over 70, haemoglobin less than 10 g/L and the Eastern Cooperative Oncology Group (ECOG) Performance Status grade 3-4 at diagnosis were associated with worse survival. Between 1985 and 2002, the Thames Cancer Registry recorded 750 cases of WM occurring in the wider area of South East England. The relative 5 year survival for patients aged less than 70 years was 70% (95% CI: 60-81%) and for patients aged 70 and over it was 50% (95% CI: 41-60%).