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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

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