title: Significant efficacy of 2-chlorodeoxyadenosine{+/-} rituximab in the treatment of splenic marginal zone lymphoma (SMZL): extended follow-up.
abstract: BACKGROUND
  Splenic marginal zone lymphoma with or without villous lymphocytes (SLVL/SMZL) is an indolent lymphoma that typically affects elderly patients and that has a median survival >10 years. It presents with marked splenomegaly. Treatment is required in symptomatic cases. Splenectomy remains one of the first-line options in patients fit for surgery. The best pharmacological strategy has not yet been identified for poor surgical risk cases. Among different possible chemotherapeutic approaches, purine analogs, alone or in association with Rituximab, seem to be a valid therapeutic choice.
  
  
  METHODS
  Fifty SMZL patients were treated with Cladribine ± anti-CD20 monoclonal antibody.
  
  
  RESULTS
  Forty-seven of 50 patients were evaluable for response. ORR was 87%: 24 of 47 patients (51%) achieved a complete hematological response (CR), 17 of 47 (36%) a partial response (PR) and 6 (13%) resulted unresponsive. Interestingly, 15 of 24 cases (62%) in CR achieved also a molecular remission. After a median follow-up of 48 months, 7 of 41 responsive cases relapsed and the 5-year PFS was 80%.
  
  
  CONCLUSIONS
  These data confirm the efficacy of this schedule emphasizing the impact of minimal residual disease even in the outcome of SMZL patients.
authors.affiliation: U.O. Hematology, Clinical and Experimental Medicine Department, University of Pisa, Italy. juliacervetti@alice.it
authors.forename: G
authors.initials: G
authors.lastname: Cervetti
authors.forename: S
authors.initials: S
authors.lastname: Galimberti
authors.forename: M
authors.initials: M
authors.lastname: Pelosini
authors.forename: F
authors.initials: F
authors.lastname: Ghio
authors.forename: N
authors.initials: N
authors.lastname: Cecconi
authors.forename: M
authors.initials: M
authors.lastname: Petrini
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000009:adverse effects / Q000627:therapeutic use*; D018951:Antigens, CD20 / Q000276:immunology*; D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017338:Cladribine / Q000009:adverse effects / Q000627:therapeutic use*; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007167:Immunotherapy; D018442:Lymphoma, B-Cell, Marginal Zone / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy; D018365:Neoplasm, Residual; D000069283:Rituximab; D013160:Splenic Neoplasms / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D018951:Antigens, CD20; D000970:Antineoplastic Agents; D017338:Cladribine; D000069283:Rituximab
keywords: MRD; cladribrine; rituximab; splenic marginal zone lymphoma