Splenic marginal zone lymphoma: a prognostic model for clinical use

The Integruppo Italiano Linfomi (IIL) carried
out a study to assess the outcomes
of splenic marginal zone lymphoma and
to identify prognostic factors in 309 patients.
The 5-year cause-specific survival
(CSS) rate was 76%. In univariate analysis,
the parameters predictive of shorter
CSS were hemoglobin levels below 12
g/dL (P < .001), albumin levels below 3.5
g/dL (P .001), International Prognostic
Index (IPI) scores of 2 to 3 (P < .001),
lactate dehydrogenase (LDH) levels above
normal (P < .001), age older than 60 years
(P .01), platelet counts below 100 000/
L (P .04), HbsAg-positivity (P .01),
and no splenectomy at diagnosis
(P .006). Values that maintained a negative
influence on CSS in multivariate analysis
were hemoglobin level less than 12
g/dL, LDH level greater than normal, and
albumin level less than 3.5 g/dL. Using
these 3 variables, we grouped patients
into 3 prognostic categories: low-risk
group (41%) with no adverse factors, intermediate-
risk group (34%) with one adverse
factor, and high-risk group (25%)
with 2 or 3 adverse factors. The 5-year
CSS rate was 88% for the low-risk group,
73% for the intermediate-risk group, and
50% for the high-risk group. The causespecific
mortality rate ( 1000 personyears)
was 20 for the low-risk group, 47
for the intermediate-risk group, and 174
for the high-risk group. This latter group
accounted for 54% of all lymphomarelated
deaths. In conclusion, with the
use of readily available factors, this prognostic
index may be an effective tool for
evaluating the need for treatment and the
intensity of therapy in an individual patient.