Mantle cell lymphoma (MCL) patients represent a difficult problem, som
etimes to establish the diagnosis but mostly because of their refracto
riness to standard lymphoma treatments. Which treatments to apply and
to whom is not yet defined. In this study, we attempted to analyze the
clinical features, to identify the major prognostic factors, and to e
valuate the outcome of 121 MCL patients treated in our institution bet
ween 1979 and 1997. Clinical data, treatment modalities, and Internati
onal Prognostic Index (IPI) score were evaluated. Median age was 63 ye
ars. Patients usually presented with advanced stage disease (87%), dis
seminated lymph nodes (57%), bone marrow involvement (79%), but with a
good performance status (PS) (81%). Lymphocytosis > 4000/mu l and/or
peripheral blood involvement was present in 36% of cases, and gastroin
testinal disease in 18%. The t(11;14)(q13;q32) and/or bcl-1 rearrangem
ent was detected in 47/57 studied cases. Median overall survival (OS)
was 3.12 years and a longer survival was significantly associated with
younger age (< 70 years), good PS (< 2), localized disease (stage I-I
I), fewer than two extra-nodal sites, absence of spleen or peripheral
blood involvement, normal serum LDH and PP-microglobulin levels, and h
emoglobin level greater than 12 g/dl. However, the IPI failed to ident
ify patients with longer OS and in a multiparametric analysis, only ol
der age, hemoglobin less than 12 g/dl, poor PS, and blood involvement
were associated with a poorer outcome. Treatment modalities had no imp
act on survival with 75% of patients relapsing or progressing. Our dat
a showed that the poor outcome of MCL patients is mainly related to ad
verse patient characteristics, a highly disseminated tumor, and some u
nknown parameters associated with the refractoriness to standard thera
py.