Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point-of-care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors and editors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

Choose from the list below to learn more about subscriptions for a:


Subscribers log in here


Treatment of marginal zone (MALT) lymphoma

INTRODUCTION

Marginal zone lymphoma (MZL) is a subtype of non-Hodgkin lymphoma (NHL) that includes three distinct diseases that have historically been classified together because they appear to arise from post-germinal center marginal zone B cells and share a similar immunophenotype:

Splenic marginal zone B cell lymphoma

Extranodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT) type (MALT-type lymphoma, MALT lymphoma)

Nodal marginal zone B cell lymphoma

This topic review will discuss the treatment of MZL. The pathogenesis, clinical manifestations, pathologic features, cytogenetics, diagnosis of these disorders is presented separately, as is their potential relationship to autoimmune disorders. (See "Clinical manifestations, pathologic features, and diagnosis of extranodal (MALT) and nodal marginal zone lymphomas" and "Clinical manifestations, pathologic features, and diagnosis of splenic marginal zone lymphoma".)

                     

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Mar 2014. | This topic last updated: Apr 16, 2014.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2014 UpToDate, Inc.
References
Top
  1. Swerdlow SH, Campo E, Harris NL, et al. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC Press, Lyon 2008.
  2. Sretenovic M, Colovic M, Jankovic G, et al. More than a third of non-gastric malt lymphomas are disseminated at diagnosis: a single center survey. Eur J Haematol 2009; 82:373.
  3. Raderer M, Wöhrer S, Streubel B, et al. Assessment of disease dissemination in gastric compared with extragastric mucosa-associated lymphoid tissue lymphoma using extensive staging: a single-center experience. J Clin Oncol 2006; 24:3136.
  4. Harada K, Murakami N, Kitaguchi M, et al. Localized ocular adnexal mucosa-associated lymphoid tissue lymphoma treated with radiation therapy: a long-term outcome in 86 patients with 104 treated eyes. Int J Radiat Oncol Biol Phys 2014; 88:650.
  5. Isobe K, Kagami Y, Higuchi K, et al. A multicenter phase II study of local radiation therapy for stage IEA mucosa-associated lymphoid tissue lymphomas: a preliminary report from the Japan Radiation Oncology Group (JAROG). Int J Radiat Oncol Biol Phys 2007; 69:1181.
  6. Tsang RW, Gospodarowicz MK, Pintilie M, et al. Stage I and II MALT lymphoma: results of treatment with radiotherapy. Int J Radiat Oncol Biol Phys 2001; 50:1258.
  7. Tsang RW, Gospodarowicz MK, Pintilie M, et al. Localized mucosa-associated lymphoid tissue lymphoma treated with radiation therapy has excellent clinical outcome. J Clin Oncol 2003; 21:4157.
  8. Son SH, Choi BO, Kim GW, et al. Primary radiation therapy in patients with localized orbital marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT Lymphoma). Int J Radiat Oncol Biol Phys 2010; 77:86.
  9. Goda JS, Gospodarowicz M, Pintilie M, et al. Long-term outcome in localized extranodal mucosa-associated lymphoid tissue lymphomas treated with radiotherapy. Cancer 2010; 116:3815.
  10. Goda JS, Le LW, Lapperriere NJ, et al. Localized orbital mucosa-associated lymphoma tissue lymphoma managed with primary radiation therapy: efficacy and toxicity. Int J Radiat Oncol Biol Phys 2011; 81:e659.
  11. Anacak Y, Miller RC, Constantinou N, et al. Primary mucosa-associated lymphoid tissue lymphoma of the salivary glands: a multicenter Rare Cancer Network study. Int J Radiat Oncol Biol Phys 2012; 82:315.
  12. Hashimoto N, Sasaki R, Nishimura H, et al. Long-term outcome and patterns of failure in primary ocular adnexal mucosa-associated lymphoid tissue lymphoma treated with radiotherapy. Int J Radiat Oncol Biol Phys 2012; 82:1509.
  13. Olszewski AJ, Desai A. Radiation Therapy Administration and Survival in Stage I/II Extranodal Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue. Int J Radiat Oncol Biol Phys 2014; 88:642.
  14. Fung CY, Tarbell NJ, Lucarelli MJ, et al. Ocular adnexal lymphoma: clinical behavior of distinct World Health Organization classification subtypes. Int J Radiat Oncol Biol Phys 2003; 57:1382.
  15. Hitchcock S, Ng AK, Fisher DC, et al. Treatment outcome of mucosa-associated lymphoid tissue/marginal zone non-Hodgkin's lymphoma. Int J Radiat Oncol Biol Phys 2002; 52:1058.
  16. Uno T, Isobe K, Shikama N, et al. Radiotherapy for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: a multiinstitutional, retrospective review of 50 patients. Cancer 2003; 98:865.
  17. Hasegawa M, Kojima M, Shioya M, et al. Treatment results of radiotherapy for malignant lymphoma of the orbit and histopathologic review according to the WHO classification. Int J Radiat Oncol Biol Phys 2003; 57:172.
  18. Suh CO, Shim SJ, Lee SW, et al. Orbital marginal zone B-cell lymphoma of MALT: radiotherapy results and clinical behavior. Int J Radiat Oncol Biol Phys 2006; 65:228.
  19. Decaudin D, de Cremoux P, Vincent-Salomon A, et al. Ocular adnexal lymphoma: a review of clinicopathologic features and treatment options. Blood 2006; 108:1451.
  20. Thieblemont C, Berger F, Dumontet C, et al. Mucosa-associated lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed. Blood 2000; 95:802.
  21. Zinzani PL, Stefoni V, Musuraca G, et al. Fludarabine-containing chemotherapy as frontline treatment of nongastrointestinal mucosa-associated lymphoid tissue lymphoma. Cancer 2004; 100:2190.
  22. Avilés A, Neri N, Calva A, et al. Addition of a short course of chemotherapy did not improve outcome in patients with localized marginal B-cell lymphoma of the orbit. Oncology 2006; 70:173.
  23. Oh SY, Ryoo BY, Kim WS, et al. Nongastric marginal zone B-cell lymphoma: analysis of 247 cases. Am J Hematol 2007; 82:446.
  24. Husain A, Roberts D, Pro B, et al. Meta-analyses of the association between Chlamydia psittaci and ocular adnexal lymphoma and the response of ocular adnexal lymphoma to antibiotics. Cancer 2007; 110:809.
  25. Ferreri AJ, Dolcetti R, Dognini GP, et al. Chlamydophila psittaci is viable and infectious in the conjunctiva and peripheral blood of patients with ocular adnexal lymphoma: results of a single-center prospective case-control study. Int J Cancer 2008; 123:1089.
  26. Chanudet E, Adam P, Nicholson AG, et al. Chlamydiae and Mycoplasma infections in pulmonary MALT lymphoma. Br J Cancer 2007; 97:949.
  27. Matthews JM, Moreno LI, Dennis J, et al. Ocular Adnexal Lymphoma: no evidence for bacterial DNA associated with lymphoma pathogenesis. Br J Haematol 2008; 142:246.
  28. Ferreri AJ, Ponzoni M, Guidoboni M, et al. Regression of ocular adnexal lymphoma after Chlamydia psittaci-eradicating antibiotic therapy. J Clin Oncol 2005; 23:5067.
  29. Ferreri AJ, Ponzoni M, Guidoboni M, et al. Bacteria-eradicating therapy with doxycycline in ocular adnexal MALT lymphoma: a multicenter prospective trial. J Natl Cancer Inst 2006; 98:1375.
  30. Govi S, Dognini GP, Licata G, et al. Six-month oral clarithromycin regimen is safe and active in extranodal marginal zone B-cell lymphomas: final results of a single-centre phase II trial. Br J Haematol 2010; 150:226.
  31. Ferreri AJ, Govi S, Pasini E, et al. Chlamydophila psittaci eradication with doxycycline as first-line targeted therapy for ocular adnexae lymphoma: final results of an international phase II trial. J Clin Oncol 2012; 30:2988.
  32. Ferreri AJ, Guidoboni M, Ponzoni M, et al. Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst 2004; 96:586.
  33. Kiesewetter B, Raderer M. Antibiotic therapy in nongastrointestinal MALT lymphoma: a review of the literature. Blood 2013; 122:1350.
  34. Grünberger B, Wöhrer S, Streubel B, et al. Antibiotic treatment is not effective in patients infected with Helicobacter pylori suffering from extragastric MALT lymphoma. J Clin Oncol 2006; 24:1370.
  35. Zucca E, Conconi A, Laszlo D, et al. Addition of rituximab to chlorambucil produces superior event-free survival in the treatment of patients with extranodal marginal-zone B-cell lymphoma: 5-year analysis of the IELSG-19 Randomized Study. J Clin Oncol 2013; 31:565.
  36. Fisher RI, Dahlberg S, Nathwani BN, et al. A clinical analysis of two indolent lymphoma entities: mantle cell lymphoma and marginal zone lymphoma (including the mucosa-associated lymphoid tissue and monocytoid B-cell subcategories): a Southwest Oncology Group study. Blood 1995; 85:1075.
  37. Raderer M, Streubel B, Woehrer S, et al. High relapse rate in patients with MALT lymphoma warrants lifelong follow-up. Clin Cancer Res 2005; 11:3349.
  38. Portlock CS, Rosenberg SA. No initial therapy for stage III and IV non-Hodgkin's lymphomas of favorable histologic types. Ann Intern Med 1979; 90:10.
  39. Horning SJ, Rosenberg SA. The natural history of initially untreated low-grade non-Hodgkin's lymphomas. N Engl J Med 1984; 311:1471.
  40. Arcaini L, Lazzarino M, Colombo N, et al. Splenic marginal zone lymphoma: a prognostic model for clinical use. Blood 2006; 107:4643.
  41. 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.
  42. Kalpadakis C, Pangalis GA, Angelopoulou MK, et al. Treatment of splenic marginal zone lymphoma with rituximab monotherapy: progress report and comparison with splenectomy. Oncologist 2013; 18:190.
  43. Else M, Marín-Niebla A, de la Cruz F, et al. Rituximab, used alone or in combination, is superior to other treatment modalities in splenic marginal zone lymphoma. Br J Haematol 2012; 159:322.
  44. Berger F, Felman P, Thieblemont C, et al. Non-MALT marginal zone B-cell lymphomas: a description of clinical presentation and outcome in 124 patients. Blood 2000; 95:1950.
  45. Catovsky D, Matutes E. Splenic lymphoma with circulating villous lymphocytes/splenic marginal-zone lymphoma. Semin Hematol 1999; 36:148.
  46. Franco V, Florena AM, Iannitto E. Splenic marginal zone lymphoma. Blood 2003; 101:2464.
  47. 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.
  48. 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.
  49. Thieblemont C, Felman P, Callet-Bauchu E, et al. Splenic marginal-zone lymphoma: a distinct clinical and pathological entity. Lancet Oncol 2003; 4:95.
  50. Olszewski AJ. Survival outcomes with and without splenectomy in splenic marginal zone lymphoma. Am J Hematol 2012; 87:E119.
  51. El Weshi A, Ribrag V, Girinski T, et al. Low and medium dose spleen radiation therapy are able to induce long-term responses in splenic lymphoma with villous lymphocytes. Br J Haematol 1998; 103:1212.
  52. Mulligan SP, Matutes E, Dearden C, Catovsky D. Splenic lymphoma with villous lymphocytes: natural history and response to therapy in 50 cases. Br J Haematol 1991; 78:206.
  53. 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. Groupe Francais d'Hématologie Cellulaire (GFHC). Br J Haematol 1996; 93:731.
  54. Suarez F, Lortholary O, Hermine O, Lecuit M. Infection-associated lymphomas derived from marginal zone B cells: a model of antigen-driven lymphoproliferation. Blood 2006; 107:3034.
  55. Saadoun D, Suarez F, Lefrere F, et al. Splenic lymphoma with villous lymphocytes, associated with type II cryoglobulinemia and HCV infection: a new entity? Blood 2005; 105:74.
  56. Hermine O, Lefrère 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.
  57. Gisbert JP, García-Buey L, Pajares JM, Moreno-Otero R. Systematic review: regression of lymphoproliferative disorders after treatment for hepatitis C infection. Aliment Pharmacol Ther 2005; 21:653.
  58. 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 multicenter Italian experience. J Clin Oncol 2005; 23:468.
  59. Brown JR, Friedberg JW, Feng Y, et al. A phase 2 study of concurrent fludarabine and rituximab for the treatment of marginal zone lymphomas. Br J Haematol 2009; 145:741.
  60. Orciuolo E, Buda G, Sordi E, et al. 2CdA chemotherapy and rituximab in the treatment of marginal zone lymphoma. Leuk Res 2010; 34:184.