Deoxycoformycin (pentostatin) in the treatment of Splenic Marginal Zone Lymphoma (SMZL)with or without villous lymphocytes

Background: Splenic marginal zone lymphoma (SMZL) is aninfrequent B-cell neoplasm that pursues an indolent course. Signs andsymptoms, mostly related to hypersplenism, are successfully managed bysplenectomy. However, the therapy of patients who are not fit for asurgical procedure or who relapse after splenectomy, is still an unsettledissue. Patients and methods: We report a phase-II study on 16 patientswith SMZL, three therapy naı¨ve and 13 pretreated, all showing systemicsymptoms or progres sive worsening of peripheral cytopenia, who weretreated with pentost atin at a dose of 4 mg/m2every oth er week for 6–10 wk. In relapsed patients, the median interval between diagnosis andtreatment was 26 month (range: 8–49). Result s: Overall, 68% of thepatients showed a clinical response. Two out three patients, who re-ceived pentostatin as first line therapy, attained a complete response(CR). One CR and seven minor or good haematological responses wererecorded in relapsed patients. Treatment toxicity, mostly haemat o-logical, proved manageable. With a median follow-up of 35 month themedian overall survival (OS) is 40 mo nth and the median progressionfree survi val (PFS) is 18 month. Conclusion : Our da ta show thatpentostatin administered every other week has a good degree of activityin the treatment of SMZL and suggest that this schedule could beconsidered a possible therapeutic option for patients who are not fit forsplenectomy or have relapsed