In this report we analyse the presenting features of a series of @entity1 diagnosed with @entity8057 ( @entity8057 ) 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 @entity1 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 @entity417 (38%), hyperviscosity (31%), B symptoms (23%), @entity548 (23%) and @entity16 (22%). Sixty-one @entity1 (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 @entity2262 ( @entity2262 ). The 83% of @entity1 who did receive treatment were distributed as follows: @entity5805 / @entity229 (43%), intermittent @entity5805 (11%), continuous @entity5805 (26%), @entity786 / @entity1891 / @entity229 (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 @entity1 presented stable and progressive disease, respectively, which was more common among @entity1 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 @entity1349 ( @entity1349 ) was 55%. The two most frequent causes of @entity204 were development of second @entity5 (31%), or @entity281 (19%). The two main variables predicting a poor @entity1349 were hyperviscosity and high @entity2262 . In summary, this study favours the use of @entity5805 -based therapy as the standard treatment for @entity8057 , and describes a subset of @entity1 who should be considered as suffering a smouldering form and who therefore do not require treatment for a long period of time.

XXXX : presenting features and outcome in a series with 217 cases.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity417 :: ('MESH:D000740', 'Disease') :: ['anaemia'] @entity548 :: ('MESH:D006470', 'Disease') :: ['bleeding'] @entity5 :: ('MESH:D009369', 'Disease') :: ['malignancies'] @entity229 :: ('MESH:D011241', 'Chemical') :: ['prednisone'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neurological symptoms'] @entity2262 :: ('567', 'Gene') :: ['beta2M', 'beta2microglobulin'] @entity786 :: ('MESH:D003520', 'Chemical') :: ['cyclophosphamide'] @entity5805 :: ('MESH:D002699', 'Chemical') :: ['chlorambucil'] @entity281 :: ('MESH:D007239', 'Disease') :: ['infections'] @entity1891 :: ('MESH:D014750', 'Chemical') :: ['vincristine'] @entity8057 :: ('MESH:D008258', 'Disease') :: ['WM', 'Waldenstr  m macroglobulinaemia'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity1349 :: ('MESH:D011475', 'Disease') :: ['OS', 'overall survival']
@entity8057 :: (MESH:D008258,Disease) :: ['Waldenstr  m macroglobulinaemia']
