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Roberto Castelli


, Splenic marginal zone lymphoma (SMZL), chronic B-cell lymphoproliferative disorder, elderly patients, Bendamustine, rituximab.


Background: Splenic marginal zone lymphoma (SMZL) is a chronic B-cell lymphoproliferative disorder, comprising less than 2% of non-Hodgkin’s lymphomas, and affecting mainly middle-aged and elderly patients with a median survival of >10 years. The typical clinical features of SMZL include splenomegaly. Treatment should be patient-tailored and can range from a ‘watchful waiting’ approach for asymptomatic patients without cytopenias to surgery, localized radiation therapy or immuno/chemotherapies. Recently, the combination of Rituximab and Bendamustine (R-Benda) has been defined as highly active in patients with follicular lymphomas, but little is known about the efficacy of R-Benda in SMZL.  

Aim of the study: The purpose of this retrospective study was to report our experience on the efficacy of R-Benda as first line treatment in 23 consecutive elderly SMZL patients. 

Results: All patients had a complete resolution of splenomegaly along with restoration of their blood counts. Nineteen patients (83%) achieved a complete response (CR) to therapy; three patients (13%) achieved a partial response (PR).Ten patients (43%) obtained molecular remission. Toxicities were mild and mainly haematological and result in dose reductions for fourteen patients. 

Conclusions: Our data suggest a high activity and good tolerance of R-Benda, despite dose reduction due to potential toxicity.


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1. Kahl, B. and Yang, D. Marginal zone lymphomas: management of nodal, splenic, and MALT NHL. Haematology 2008 Am Soc Haematology EducProgram: 359–364.
2. Zucca, E ,Dreyling, M. and Grp, E.G.W. Gastric marginal zone lymphoma of malt type: esmo clinical practice guidelines for diagnosis, treatment and follow-up. Annals of Oncology2010 21: v175-v176.
3. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood 1994; 84:1361–1392.
4. Troussard, X., Valensi, F., Duchayne, E., Garand, R., Felman, P., Tulliez, M. et al. Splenic lymphoma with villous lymphocytes: clinical presentation, biology and prognostic factors in a series of 100 patients. GroupeFrancaisd’HematologieCellulaire (GFHC). Br J Haematol 1996 93: 731–736.
5. Castelli R, Wu MA, Arquati M, Zanichelli A, Suffritti C, Rossi D, Cicardi M. High prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibitor deficiency. Br J Haematology. 2016 Jan 5. doi: 10.1111/bjh.13908. [Epub ahead of print]
6. Berger, F, Felman, P, Thieblemont, C., Pradier, T., Baseggio, L., Bryon, P. et al. Non-MALT marginal zone B-cell lymphomas: a description of clinical presentation and outcome in 124 patients. Blood 200095: 1950–1956.
7. Cervetti, G., Mechelli, S., Riccioni, R., Galimberti, S., Caracciolo, F. and Petrini, M. (2005) High efficacy of rituximab in indolent HCV-related lymphoproliferative disorders associated with systemic autoimmunediseases. ClinExpRheumatol2005 23: 877–880.
8. Zucca E, Conconi A, Laszlo D et al Addition ofrituximab to chlorambucil produces superior event-free survival in the treatment of patients with extranodal marginal-zone B-cell lymphoma: 5-year analysis if the IELSG-19 randomized study. J ClinOncol (2013) 31:565–72
9. Ben Simon GJ, Cheung N,McKelvie P et al (2006) Oral clorambucil for extranodal, marginal zone, B-cell lymphoma if mucosa-associatedlymphoid tissue of the orbit. Ophthalmology 113:1209–13
10. TrochM,Kiesewetter B, Willenbacher Wet al Rituximab plus subcutaneous cladribine in patients with extranodal marginal zone Bcelllymphoma of mucosa-associated lymphoid tissue: a phase II .Haematologica 2013: 98:264–8
11. Kalpadakis, C., Pangalis, G, Dimopoulou, M., Vassilakopoulos, T., Kyrtsonis, M., Korkolopoulou, P. et al. Rituximab monotherapy is highly effective in splenic marginal zone lymphoma. HematolOncol 2007 l25:127–131
12. EnnishiD, Yokoyama M, Mishima Y et al Rituximab plus CHOP as an initial chemotherapy for patients with disseminated MALT lymphoma. Leuk Lymphoma 2007. 48:2241–3.
13. Derksen PWB, Langerak AW, Kerkhof E, et al. Comparison ofdifferent polymerase chain reaction-based approaches for clonalityassessment of immunoglobulin heavy-chain gene rearrangementsin B-cell neoplasia. Mod Pathol 1999; 12: 794–805.
14. Arcaini, L., Lazzarino, M., Colombo, N., Burcheri, S., Boveri, E., Paulli, M. et al. (2006) Splenicmarginal zone lymphoma: a prognostic model for clinical use. Blood 2006 107: 4643–4649
15. Arcaini, L. and Paulli, M. (2010) Splenic marginal zone lymphoma: hydra with many heads?Haematologica95: 534–537
16. Franco V, Florena AM, Stella M, et al. Splenectomy influencesbone marrow infiltration in patients with splenic marginal zone cell lymphoma with or without villous lymphocytes. Cancer2001; 91: 294–301.
17. Bennett, M. and Schechter, G. Treatment of splenic marginal zone lymphoma: splenectomy versus rituximab. SeminHematol(2010) 47: 143–147.
18. Troussard X, Valensi F, Duchayne E, et al. Splenic lymphoma with villous lymphocytes. Clinical presentation, biology andprognostic factors in a series of 100 patients. Br J Haematol 1996; 93: 731–736.
19. Catovsky D, Matutes E. Splenic lymphoma with circulating villous lymphocytes/ splenic marginal-zone lymphoma. SeminHematol 1999; 36: 148–154
20. Lefrere F, Hermine O, Belanger C, et al. Fludarabine: an effective treatment in patients with splenic lymphoma with villous lymphocytes. Leukemia 2000; 14: 573–575.
21. Pangalis GA, Vassilakopoulos TP, KalpadakisCh, et al. Treatment of indolent lymphomas from watch and wait to high dose therapy. Hematology 2005; 1: 6–9.
22. Riccioni R, Caracciolo F, Galimberti S, Cecconi N, Petrini M. Low dose 2-CdA schedule activity in splenicmarginal zone lymphomas. HematolOncol 2003; 21: 163–168.
23. Lefrerre F, Hermine O, Francois S. et al. Lack of efficacy of 2-chlorodeoxyadenoside in the treatment of splenic lymphoma with villous lymphocytes. Leuk Lymphoma 2000; 40: 113– 117
24. Bolam S, Orchard J, Oscier D. Fludarabine is effective in the treatment of splenic lymphoma with villous lymphocytes. Br J Haematol 1997; 99: 158–161
25. Damaj G, Gressin R, Bouabdallah K, Cartron G, Choufi B, Gyan E, et al. Results from a prospective, open-label, phase II trial of Bendamustine in refractory or relapsed T-cell lymphomas: the BENTLY trial. J ClinOncol 2013;31:104–10)
26. Rummel MJ, Gregory SA. Bendamustine's emerging role in the management of lymphoid malignancies. SeminHematol 2011;48 Suppl 1: S24–36);
27. Chacar C, Jabbour E, Ravandi F, Borthakur G, Kadia T, Estrov Z, et al. Phase I-II study of Bendamustine in patients with acute leukemia and high risk myelodysplastic syndrome. Clin Lymphoma Myeloma Leuk 2012;12:197–200
28. Kahl, B., Bartlett, N., Leonard, J., Chen, L., Ganjoo, K., Williams, M. et al. (2010a) Bendamustine is effective therapy in patients with rituximab-refractory, indolent B-cell non-Hodgkin lymphoma: results from a multicenter study. Cancer 116: 106–114.
29. Kiesewetter B, Mayerhoefer ME, Lukas J, Zielinski CC, Müllauer L, Raderer M . Rituximab plus Bendamustine is active in pretreated patients with extragastric marginal zone B cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma Ann Hematol. 2014 Feb;93(2):249-53).