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 relapse

Response-guided ABVD chemotherapy plus involved-field radiation therapy for intermediate-stage hodgkin lymphoma in the pre-positron emission tomography era: A Gruppo Italiano Studio Linfomi (GISL) prospective trial

Purpose: In the pre-positron emission tomography era, the Gruppo Italiano Studio Linfomi (GISL) investigated the feasibility and efficacy of a treatment based on a response-tailored number of doxorubicin/bleomycin/vinblastine/ dacarbazine (ABVD) courses in 218 intermediate-stage Hodgkin lymphoma patients. Patients and Methods: Patients with stage I/II showing at least one adverse prognostic factor and stage IIIA without adverse prognostic factors were recruited. Treatment included a first step of 3 ABVD courses, followed by an early-restaging. Patients in CR/CRu received 1 additional ABVD cycle, patients in PR received 3 more ABVD, and nonresponder patients went off study. Involved-field radiation therapy (RT) was recommended on chemotherapy completion. Results: The median age was 30 years (range, 15-68 years) and 131 patients (61%) were female. Seven percent of patients were in stage I, 78% in stage II, and 15% in stage III; B-symptoms, bulky tumor and erythrocyte sedimentation rate > 30 were recorded in 20%, 26%, and 43% of cases, respectively. The CR/CRu rate was 62% at early restaging, 72% at the end of chemotherapy, and 95% following RT. With a median follow-up of 74 months (range, 6-193 months), 7-year overall survival, relapse-free survival, and freedom from treatment failure were 91.8% (95% CI, 86%-95.5%), 89.2% (95% CI, 82.8%-93.3%), and 81.8% (95% CI, 75.2%-86.7%), respectively. Patients in CR/CRu on early restaging, receiving 4 ABVD, had an excellent outcome with 7-year RFS and cause-specific survival similar to those of the late responders treated with 6 ABVD (RFS, 87.5% vs. 90.5% and CSS, 96.6% vs. 92.7%, respectively). Conclusion: The response-guided ABVD program we report, based on standard clinical staging procedures, proved to be feasible and safe in patients with intermediate-stage Hodgkin lymphoma