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

2001; Wiley; Volume: 115; Issue: 3 Linguagem: Inglês

10.1046/j.1365-2141.2001.03144.x

1365-2141

Ramón García‐Sánz, Silvia Montoto, Agustín Torrequebrada, Alfonso García de Coca, J Petit, Anna Sureda, José Antonio Rodríguez‐García, Pilar Massó, Ana Pérez‐Aliaga, María Dolores Monteagudo, Isabel Navarro, Gemma Moreno, Carmen Toledo, Aránzazu Alonso, Carles Besses, Joan Besalduch, Isidro Jarque, Perla Salama, José‐Ángel Hernández‐Rivas, Blanca Navarro, Joan Bladé, Jesús F. San Miguel,

Neuroendocrine Tumor Research Advances

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 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 β 2 microglobulin (β2M). 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 β2M. 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.