Haematologica 2003 - Supplements
Haematologica 2003 - Supplements
Haematologica 2003 - Supplements
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Aim: To identify predictor features of malignant transformation<br />
in a large series of patients with MGUS and prolonged follow-up.<br />
Patients and Methods: Four hundred and thirty-four patients (200<br />
M/234 F; median age: 66 yrs) diagnosed with MGUS in a single<br />
institution from September 1970 to January 2001 with a<br />
minimum follow-up of one year were included in the study. All<br />
patients had a M-protein size 15 g/L) as well as the<br />
percentage of bone marrow plasma cells (i.e. > 5 %) predicted<br />
malignant transformation.<br />
080<br />
CLINICAL EVOLUTION AND PROGNOSTIC FACTORS<br />
IN 300 PATIENTS WITH ASYMPTOMATIC IgM<br />
MONOCLONAL GAMMOPATHY<br />
M.Goldaniga, P.Gobbi, S.Cortelazzo, C.Broglia, A.Guffanti,<br />
E.Oldani, C.Stelitano, B.Bronzino, R.Calori, E.Pogliani,<br />
F.Merli and L.Baldini<br />
1) GISL (Gruppo Italiano Studio Linfomi) 2) Divisione di<br />
Ematologia, Ospedali Riuniti , Bergamo, Italy<br />
Asymptomatic clonal macroglobulinemia (ACM) is currently<br />
classified as an IgM MGUS or indolent Waldenström’s<br />
macroglobulinemia (WM), but its clinical relevance and<br />
propensity to evolve into lymphoid neoplasms is not well defined.<br />
We retrospectively evaluated 300 patients with ACM in order to<br />
identify the clinico–pathological features relating to its evolution<br />
into a symptomatic lymphoid neoplasm requiring treatment and<br />
create a prognostic score capable of distinguishing patient<br />
subgroups with different prognoses. The exclusion criteria were:<br />
1) treatment-requiring conditions (high or rapidly increasing<br />
serum IgM levels, hyperviscosity or systemic symptoms,<br />
organomegaly and/or cytopenia); 2) the presence of an<br />
autoimmune disorder, HCV-related cryoglobulinemia,<br />
amyloidosis or other complications due to tissue deposits of IgM<br />
or clonal IgM leading to MM or low grade nonlymphoplasmacytic<br />
lymphoma; or 3) cases with an uncertain<br />
follow-up or unclear disease evolution or treatment criteria. The<br />
main characteristics at diagnosis are shown in the table.<br />
Variable No. of Value<br />
evaluated<br />
pts<br />
Age in years (mean ± 300 63 ± 11<br />
SD)<br />
M/F (ratio) 300 186/114<br />
Serum MC g/dL 300 1.1 (0.11- 3.0)<br />
(median, min-max)<br />
Hb g/dL (median, 300 13.8 (11.0-17.9)<br />
min-max)<br />
Peripheral<br />
287 2 (0.4-3.6)<br />
lymphocytes x 109/L<br />
(median, min-max)<br />
PLT x 109/L (median, 298 238 (100-627)<br />
min-max)<br />
Serum LDH U/l 187 319 ± 104 (134 -986)<br />
(mean ± SD, minmax)<br />
Serum β2<br />
132 2.504 ± 1.052 (195-7.962)<br />
microglobulin µg/ml<br />
(mean ± SD, minmax)<br />
No.of pts. with 296 21/296 ( 7 )<br />
detectable Bence<br />
Jones proteinuria (%)<br />
No.of pts, with one 258 34/258 ( 13.2 )<br />
serum polyclonal Ig<br />
reduction (%)<br />
Bone marrow<br />
lymphoplasma cells<br />
% (median, min-max)<br />
237 11 ( 3 - 92 )<br />
After a median follow-up of 55 months (6-221), 43/300 patients<br />
(14.7%) required chemotherapy for symptomatic WM (70%),<br />
NHL (18.5%), amyloidosis (7%) or peripheral neuropathy<br />
(4.5%). Five- and 10-year overall survival was 98% and 90%,<br />
and evolution-free-survival 91% and 78%.<br />
The features correlating with evolution to overt<br />
lymphoproliferative disease were: serum clonal IgM<br />
concentration (P