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Splenic marginal zone lymphoma with and without villous lymphocytes

  • Chronic Leukemia
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Opinion statement

Splenic marginal zone lymphoma with or without villous lymphocytes (SLVL/SMZL) is a low grade B-cell lymphoma that affects patients in the sixth decade and has a median survival greater than 10 years. A substantial proportion of patients die from causes unrelated to the disease. Close to a third of the patients do not require intervention and a policy of watch and see is reasonable and recommended. There are several therapeutic options that have proved effective in these patients. Due to the natural history of the lymphoma, the main goal of all these treatments is to achieve control of the disease rather than its eradication. Retrospective designs of all documented studies, the lack of uniform response criteria and the heterogeneity in the patient’s features makes interpretation of the data difficult. Splenectomy remains one of the first line options in patients fit for surgery. Amongst chemotherapy, purine analogues, in particular fludarabine in combination or not with Rituximab and Rituximab alone have a greater efficacy than alkylating agents in terms of achieving better quality of response and longer progression free survival; therefore these agents are recommended particularly in patients who are not candidates for surgery or relapse after splenectomy. In the small proportion of patients with concomitant hepatitis C virus (HCV) infection, Interferon-alpha, ribavirin or a combination of both has demonstrated a significant activity with responses correlating with clearance of HCV RNA in the blood; therefore, these agents should be considered in the therapeutic scenario as a first line in these small cohort of patients. Patients that transform to high-grade lymphoma and the minority that have TP53 abnormalities should be treated with other schedules. Prospective randomized trials would be desirable to ascertain the independent prognostic factors and the biological features that predict disease progression and drug resistance to device the optimal management and treatment for SLVL/SMZL.

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References and Recommended Reading

Paper of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance.

  1. Isaacson PG, Piris MA, Catovsky D, et al.: Splenic marginal zone cell lymphoma. In: Jaffe ES, Harris N, Vardiman JW editors. Tumours of Haemopoietic and lymphoid tissues. World Health Organization Classification of tumours Lyon, France: IARC Press; 2001, 135–137

  2. Catovsky D, Matutes E: Splenic lymphoma with circulating villous lymphocytes/splenic marginal zone lymphoma. Semin Hematol 1999, 36:148–154

    Google Scholar 

Comment: A review of the natural history of the disease, diagnostic criteria, prognosis and therapeutic options

  1. Arcani L Paulli M, Boveri E, et al.: Splenic and nodal marginal zone lymphomas are indolent disorders at high hepatitis C virus seroprevalence with distinct presenting features but similar morphologic and phenotypic features. Cancer 2004, 100:107–115

    Google Scholar 

  2. Mele A, Pulsoni A, Bianco E, et al.: Hepatitis C virus and B-cell non-Hodgkin lymphomas: an Italian multicenter case-control study. Blood 2003, 102:996–999.

    Article  PubMed  CAS  Google Scholar 

  3. Isaacson PG, Matutes E, Burke M, Catovsky D The histopathology of splenic lymphoma with villous lymphocytes. Blood 1994, 84:3828–3834.

    PubMed  CAS  Google Scholar 

  4. Mateo M, Mollejo M, Villuendas R, et al.: 7q31–32 allelic loss is a frequent finding in splenic marginal zone lymphoma. Am J Pathol 1999, 154:1583–1589.

    PubMed  CAS  Google Scholar 

  5. Gruszka-Westwood AM, Matutes E, Coignet LJA, et al.: The incidence of trisomy 3 in splenic lymphoma with villous lymphocytes: a study by FISH. Br J Haematol 1999;104:600–604.

    Article  PubMed  CAS  Google Scholar 

  6. Gruszka-Westwood AM, Hamoudi RA, Matutes E, et al.: p53 abnormalities in splenic lymphoma with villous lymphocytes (SLVL). Blood 2003; 97:3552–3558.

    Article  Google Scholar 

  7. Thieblemont C, Felman P, Callet-Bauchu E, et al.: Splenic marginal zone lymphoma: a distinct clinical and pathological entity. The Lancet Oncol 2003; 4:95–103

    Google Scholar 

  8. Arcaini L, Lazzarino M, Colombo N et al.: Splenic marginal zone lymphoma: a prognostic model for clinical use. Blood 2006, 107:4643–4649

    Google Scholar 

  9. Chacon J, Mollejo M, Munoz E et al.: Splenic marginal zone lymphoma: clinical characteristics and prognostic factors in a series of 60 patients. Blood 2002, 100:1648–1654

    Google Scholar 

  10. Troussard X, Valensi F, Duchayne E, et al.: Splenic lymphoma with villous lymphocytes. Clinical presentation, biology and prognostic factors in a series of 100 patients. Br J Haematol. 1996; 93:731–736.

    Article  PubMed  CAS  Google Scholar 

  11. Iannitto E, Ambrosetti A, Ammatuna E, et al.: Splenic marginal zone lymphoma with or without villous lymphocytes. Hematologic findings and outcomes in a series of 57 patients. Cancer 2004; 101:2050–2057.

    Article  PubMed  Google Scholar 

  12. Parry-Jones N, Matutes E, Gruszka-Westwood AM, et al.: Prognostic features of splenic lymphoma with villous lymphocytes: a report on 129 patients. Br J Haematol 2003, 120: 759–764

    Google Scholar 

  13. Camacho FI, Mollejo M, Mateo MS, et al.: Progression to large B-cell lymphoma in splenic marginal zone lymphoma: a description of a series of 12 cases. Am J Surg Pathol 2001, 25:1268–1276

    Google Scholar 

  14. Mulligan SP, Matutes E, Dearden C, et al.: Splenic lymphoma with villous lymphocytes: natural history and response to therapy in 50 cases. Br J Haematol 1991;78:206–209.

    PubMed  CAS  Google Scholar 

  15. Franco V, Florena AM, Stella M, et al.: Splenectomy influences bone marrow infiltration in patients with splenic marginal zone lymphoma with or without villous lymphocytes. Cancer 2001; 91:294–301.

    Article  PubMed  CAS  Google Scholar 

  16. 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.

    Article  PubMed  CAS  Google Scholar 

  17. Yasukawa M, Yamaguchi H, Azuma T, et al.: Dramatic efficacy of fludarabine in the treatment of an aggressive case of splenic lymphoma with villous lymphocytes. Eur J Haematol 2002; 69:112–114.

    Article  PubMed  Google Scholar 

  18. Lefrere F, Levy V, Francois S, et al.: Fludarabine treatment in patients with splenic lymphoma with villous lymphocytes: an update. Leukemia 2004, 18:1924–1925

    Google Scholar 

  19. Virchis A, Mehta A: Splenic lymphoma with villous lymphocytes (SLVL) responding to 2-chlorodeoxyadenosine (2-CDA). Br J Haematol 1998; 100:609.

    Article  PubMed  CAS  Google Scholar 

  20. Riccioni R, Caracciolo F, Galimberti S, et al.: Low dose 2-CDA schedule activity in splenic marginal zone lymphomas. Hematol Oncol 2003; 21:163–168.

    Article  PubMed  CAS  Google Scholar 

  21. Lefrere F, Hermine O, Francois S, et al.: Lack of efficacy of 2-chlorodeoxyadenosine in the treatment of splenic lymphoma with villous lymphocytes. Leuk Lymphoma 2000, 40:113–117.

    Article  PubMed  CAS  Google Scholar 

  22. Delanoy A: 2 chlorodeoxyadenosine: clinical applications in hematology. Blood Rev 1996, 10:148–166.

    Article  Google Scholar 

  23. Iannitto E, Minardi V, Calvaruso G, et al.: Deoxycoformycin (penttostatin) in the treatment of splenic marginal zone lymphoma (SMZL) with or without villous lymphocytes. Eur J Haematol 2005, 75:130–135.

    Article  PubMed  CAS  Google Scholar 

  24. Fabbri A, Gozetti A, Lazzi S, et al.: Activity of rituximab in refractory splenic marginal zone lymphoma complicated with autoimmune hemolytic anemia. Clin Lymphoma Myeloma 2006, 6:496–499.

    Article  PubMed  Google Scholar 

  25. Bennett M, Sharma K, Yegena S, et al.: Rituximab monotherapy for splenic marginal zone lymphoma. Haematologica 2005, 90:856–858

    Google Scholar 

  26. Arcaini L, Orlandi E, Scotti M, et al.: Combination of rituximab, cyclophosphamide and vincristine induces complete hematologic remission of splenic marginal zone lymphoma. Clin Lymphoma 2004; 4:250–252.

    Article  PubMed  CAS  Google Scholar 

  27. Tsimberidou AM, Catovsky D, Schlette E, et al.: Outcomes in patients with splenic marginal zone lymphoma and marginal zone lymphoma treated with Rituximab with or without chemotherapy or chemotherapy alone. Cancer 2006, 107:125–135

    Google Scholar 

Comment: Retrospective study demonstrating the efficacy of Rituximab alone or combined with chemotherapy

  1. Hermine O, Lefrere F, Bronowicki JP, et al.: Regression of splenic lymphoma with villous lymphocytes after treatment of hepatitis C virus infection. N Engl J Med 2002, 347:89–94

    Google Scholar 

Comment: Seminal report describing the efficacy of IFN plus or nor ribavirin in SLVL/SMZL associated to hepatitis C virus infection

  1. Vallisa D, Bernuzzi P, Arcaini L, et al.: Role of anti-hepatitis C virus (HCV) treatment in HCV-related low grade B-cell non-Hodgkin’s lymphoma: A muticenter Italian Experience. J Clin Oncol 2005, 23:468–473.

    Article  PubMed  CAS  Google Scholar 

  2. Keladi C, Rollor F, Park S, et al.: Response to antiviral treatment in hepatitis C virus associated marginal zone lymphomas. Leukemia 2004, 18:1711–1716.

    Article  CAS  Google Scholar 

  3. Osuji NC, del Guidice I, Matutes E, et al.: The efficacy of alemtuzumab for refractory chronic lymphocytic leukemia in relation to cytogenetic abnormalities of p53. Haematologica 2005, 90:1435–1436.

    PubMed  CAS  Google Scholar 

  4. Thornton PD, Matutes E, Bosanquet AG, et al.: High dose methylprednisolone can induce remissions in CLL patients with p53 abnormalities. Ann. Haematol 2003; 82:759–765.

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Haemato-Oncology Unit, Royal Marsden Hospital and Institute of Cancer Research, 203 Fulham Road, London, SW3 6JJ, UK

    Estella Matutes MD, PhD, FRCPath

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  1. Estella Matutes MD, PhD, FRCPath
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Correspondence to Estella Matutes MD, PhD, FRCPath.

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Matutes, E. Splenic marginal zone lymphoma with and without villous lymphocytes. Curr. Treat. Options in Oncol. 8, 109–116 (2007). https://doi.org/10.1007/s11864-007-0026-0

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