Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases

Br J Haematol. 2001 Dec;115(3):575-82. doi: 10.1046/j.1365-2141.2001.03144.x.

Abstract

In this report we analyse the presenting features of a series of patients diagnosed with Waldenström macroglobulinaemia (WM) in Spain over the last 10 years. Criteria for diagnosis required a serum monoclonal IgM protein > or = 30 g/l and > 20% bone marrow lymphocytes. Two hundred and seventeen patients were included in the study, with a median age of 69 years and male/female ratio of 2:1. The most common symptoms at diagnosis were anaemia (38%), hyperviscosity (31%), B symptoms (23%), bleeding (23%) and neurological symptoms (22%). Sixty-one patients (27%) were asymptomatic at diagnosis and, to date, 32 of them have not received chemotherapy. Variables predicting a shorter survival free of therapy were haemoglobin < 12.5 g/dl and high beta2microglobulin (beta2M). The 83% of patients who did receive treatment were distributed as follows: chlorambucil/prednisone (43%), intermittent chlorambucil (11%), continuous chlorambucil (26%), cyclophosphamide/vincristine/prednisone (COP, 13.5%) and other (6.5%). Response to therapy was complete in 2%, partial in 48% and minor in 10%. Finally, 28% and 13% of patients presented stable and progressive disease, respectively, which was more common among patients treated with COP. Progression-free survival was 43% at 5 years, with three independent predictors for shorter progression-free survival (PFS): COP treatment, age > 65 and B symptoms at diagnosis. The 10-year projected overall survival (OS) was 55%. The two most frequent causes of death were development of second malignancies (31%), or infections (19%). The two main variables predicting a poor OS were hyperviscosity and high beta2M. In summary, this study favours the use of chlorambucil-based therapy as the standard treatment for WM, and describes a subset of patients who should be considered as suffering a smouldering form and who therefore do not require treatment for a long period of time.

Publication types

  • Review

MeSH terms

  • Aged
  • Antibodies, Anti-Idiotypic / analysis*
  • Antibodies, Monoclonal / blood
  • Antineoplastic Agents, Alkylating / therapeutic use
  • Chlorambucil / therapeutic use
  • Female
  • Humans
  • Immunoglobulin M / immunology*
  • Incidence
  • Lymphocyte Count
  • Lymphocytes / blood*
  • Male
  • Middle Aged
  • Prognosis
  • Spain / epidemiology
  • Survival Rate
  • Treatment Outcome
  • Waldenstrom Macroglobulinemia / diagnosis*
  • Waldenstrom Macroglobulinemia / epidemiology
  • Waldenstrom Macroglobulinemia / therapy*
  • beta 2-Microglobulin / analysis

Substances

  • Antibodies, Anti-Idiotypic
  • Antibodies, Monoclonal
  • Antineoplastic Agents, Alkylating
  • Immunoglobulin M
  • beta 2-Microglobulin
  • Chlorambucil