X. Troussard et al., SPLENIC LYMPHOMA WITH VILLOUS LYMPHOCYTES - CLINICAL PRESENTATION, BIOLOGY AND PROGNOSTIC FACTORS IN A SERIES OF 100 PATIENTS, British Journal of Haematology, 93(3), 1996, pp. 731-736
The diagnosis of splenic lymphoma with villous lymphocytes (SLVL) was
assessed by a panel of cytologists in a series of 100 patients. Clinic
al and biological characteristics were analysed in relation to prognos
is. SLVL is a chronic B-cell lymphoproliferative disorder characterize
d by splenomegaly and the presence, in peripheral blood, of lymphocyte
s with 'villous' projections, The cytological diagnosis can be difficu
lt in patients without an absolute lymphocytosis which was observed in
24% of cases. B-ceIls expressed CD19(+), CD20(+), CD22+, CD24(+) and
DBA44(+), whereas the expression of CD5, CD10 and CD25 was usually neg
ative. SLVL is a disease of the elderly with a relatively benign clini
cal course. In the present series the 5-year overall survival was 78%,
Deaths in 15 patients were related to disease progression or treatmen
t (nine cases), Patients with a leucocyte count >30 x 10(9)/l or lymph
ocyte count <4 x 10(9)/l or initially treated with chemotherapy had si
gnificantly (P < 0.001) lower overall survival than other patients. Fr
om these findings, treatment abstention should be considered in patien
ts with favourable prognostic factors; on the other hand, the efficacy
of conventional chemotherapy remains to be evaluated in patients with
unfavourable prognostic factors.