Splenic marginal zone lymphoma medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Muhammad Affan M.D.[2], Sowminya Arikapudi, M.B,B.S. [3]

Overview

There is no standardized treatment of splenic marginal zone lymphoma (SMZL) The optimal therapy depends on the clinical presentation. Asymptomatic patients may only be observed routinely without any treatment as it is an indolent tumor. Symptomatic patients may treated with either surgery, immunotherapy, chemotherapy, immunochemotherapy or antiviral drugs. Both surgery and immunotherapy are equally effective but recently immunotherapy is considered as a better treatment option as there is no risk of complications that are associated with surgery.

Medical Therapy

The optimal therapy for splenic marginal zone lymphoma (SMZL) depends on the clinical presentation. Treatment options for the condition are as under:

Watchful waiting

It is a slow growing tumor so patients may not need treatment right away unless symptomatic. Asymptomatic patients may be observed every 3-6 months.^[1]

Immunotherapy

In the pre-rituximab era splenectomy was considered to be the treatment of choice for splenic marginal zone lymphoma and still is performed for its treatment but rituximab is reported to be more beneficial in old age patients with co-morbid conditions.^[2]
Rituximab was found to be equally effective when compared to splenectomy for the treatment of splenic marginal zone lymphoma (SMZL) in terms of complete remission and overall survival of the patient with no complications of the surgical procedure.^[3]^[4]^[5]^[6]^[7]

Chemotherapy

chemotherapeutic drugs such as cyclophosphamide, chlorambucil have been used in the past for the treatment of splenic marginal zone lymphoma but the results were not favorable.^[8].
Purine analogues such as fludarabine, pentostatin, have also been used. They are superior to alkylating agents in terms of efficacy but not better than rituximab and in addition to that they have their side effects as well.^[9]

Immunochemotherapy

Several chemotherapy regimens have been used in combination with ‘rituximab for the treatment of splenic marginal zone lymphoma (SMZL) showing high response rates and prolonged duration of response than chemotherapy alone.^[10]^[2]
But addition of chemotherapy doesn’t seem to improve the efficacy of rituximab.^[7]

Antiviral therapy

Patients with splenic marginal zone lymphoma (SMZL) and hepatitis C infection who do not require immediate cyto-reductive therapy should be treated with antiviral therapy as it results in tumor regression in majority of the cases.^[11]^[12]

References

↑ Matutes E, Oscier D, Montalban C, Berger F, Callet-Bauchu E, Dogan A, Felman P, Franco V, Iannitto E, Mollejo M, Papadaki T, Remstein ED, Salar A, Solé F, Stamatopoulos K, Thieblemont C, Traverse-Glehen A, Wotherspoon A, Coiffier B, Piris MA (March 2008). “Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria”. Leukemia. 22 (3): 487–95. doi:10.1038/sj.leu.2405068. PMID 18094718.
↑ ^2.0 ^2.1 Tsimberidou AM, Catovsky D, Schlette E, O’Brien S, Wierda WG, Kantarjian H, Garcia-Manero G, Wen S, Do KA, Lerner S, Keating MJ (July 2006). “Outcomes in patients with splenic marginal zone lymphoma and marginal zone lymphoma treated with rituximab with or without chemotherapy or chemotherapy alone”. Cancer. 107 (1): 125–35. doi:10.1002/cncr.21931. PMID 16700034.
↑ Bennett M, Schechter GP (April 2010). “Treatment of splenic marginal zone lymphoma: splenectomy versus rituximab”. Semin. Hematol. 47 (2): 143–7. doi:10.1053/j.seminhematol.2010.01.004. PMID 20350661.
↑ Kalpadakis C, Pangalis GA, Angelopoulou MK, Sachanas S, Kontopidou FN, Yiakoumis X, Kokoris SI, Dimitriadou EM, Dimopoulou MN, Moschogiannis M, Korkolopoulou P, Kyrtsonis MC, Siakantaris MP, Papadaki T, Tsaftaridis P, Plata E, Papadaki HE, Vassilakopoulos TP (2013). “Treatment of splenic marginal zone lymphoma with rituximab monotherapy: progress report and comparison with splenectomy”. Oncologist. 18 (2): 190–7. doi:10.1634/theoncologist.2012-0251. PMC 3579603. PMID 23345547.
↑ Perrone S, D’Elia GM, Annechini G, Ferretti A, Tosti ME, Foà R, Pulsoni A (May 2016). “Splenic marginal zone lymphoma: Prognostic factors, role of watch and wait policy, and other therapeutic approaches in the rituximab era”. Leuk. Res. 44: 53–60. doi:10.1016/j.leukres.2016.03.005. PMID 27030961.
↑ Olszewski AJ, Ali S (March 2014). “Comparative outcomes of rituximab-based systemic therapy and splenectomy in splenic marginal zone lymphoma”. Ann. Hematol. 93 (3): 449–58. doi:10.1007/s00277-013-1900-4. PMID 24057925.
↑ ^7.0 ^7.1 Else M, Marín-Niebla A, de la Cruz F, Batty P, Ríos E, Dearden CE, Catovsky D, Matutes E (November 2012). “Rituximab, used alone or in combination, is superior to other treatment modalities in splenic marginal zone lymphoma”. Br. J. Haematol. 159 (3): 322–8. doi:10.1111/bjh.12036. PMID 23016878.
↑ Troussard X, Valensi F, Duchayne E, Garand R, Felman P, Tulliez M, Henry-Amar M, Bryon PA, Flandrin G (June 1996). “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. 93 (3): 731–6. PMID 8652403.
↑ Bolam S, Orchard J, Oscier D (October 1997). “Fludarabine is effective in the treatment of splenic lymphoma with villous lymphocytes”. Br. J. Haematol. 99 (1): 158–61. PMID 9359516.
↑ Cervetti G, Galimberti S, Pelosini M, Ghio F, Cecconi N, Petrini M (September 2013). “Significant efficacy of 2-chlorodeoxyadenosine{+/-} rituximab in the treatment of splenic marginal zone lymphoma (SMZL): extended follow-up”. Ann. Oncol. 24 (9): 2434–8. doi:10.1093/annonc/mdt181. PMID 23712547.
↑ Arcaini L, Vallisa D, Rattotti S, Ferretti VV, Ferreri AJ, Bernuzzi P, Merli M, Varettoni M, Chiappella A, Ambrosetti A, Tucci A, Rusconi C, Visco C, Spina M, Cabras G, Luminari S, Tucci M, Musto P, Ladetto M, Merli F, Stelitano C, d’Arco A, Rigacci L, Levis A, Rossi D, Spedini P, Mancuso S, Marino D, Bruno R, Baldini L, Pulsoni A (July 2014). “Antiviral treatment in patients with indolent B-cell lymphomas associated with HCV infection: a study of the Fondazione Italiana Linfomi”. Ann. Oncol. 25 (7): 1404–10. doi:10.1093/annonc/mdu166. PMID 24799461.
↑ Rossotti R, Travi G, Pazzi A, Baiguera C, Morra E, Puoti M (January 2015). “Rapid clearance of HCV-related splenic marginal zone lymphoma under an interferon-free, NS3/NS4A inhibitor-based treatment. A case report”. J. Hepatol. 62 (1): 234–7. doi:10.1016/j.jhep.2014.09.031. PMID 25285757.

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[pmid18094718-1] Matutes E, Oscier D, Montalban C, Berger F, Callet-Bauchu E, Dogan A, Felman P, Franco V, Iannitto E, Mollejo M, Papadaki T, Remstein ED, Salar A, Solé F, Stamatopoulos K, Thieblemont C, Traverse-Glehen A, Wotherspoon A, Coiffier B, Piris MA (March 2008). “Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria”. Leukemia. 22 (3): 487–95. doi:10.1038/sj.leu.2405068. PMID 18094718.

[pmid16700034-2] 2.0 ^2.1 Tsimberidou AM, Catovsky D, Schlette E, O’Brien S, Wierda WG, Kantarjian H, Garcia-Manero G, Wen S, Do KA, Lerner S, Keating MJ (July 2006). “Outcomes in patients with splenic marginal zone lymphoma and marginal zone lymphoma treated with rituximab with or without chemotherapy or chemotherapy alone”. Cancer. 107 (1): 125–35. doi:10.1002/cncr.21931. PMID 16700034.

[pmid20350661-3] Bennett M, Schechter GP (April 2010). “Treatment of splenic marginal zone lymphoma: splenectomy versus rituximab”. Semin. Hematol. 47 (2): 143–7. doi:10.1053/j.seminhematol.2010.01.004. PMID 20350661.

[pmid23345547-4] Kalpadakis C, Pangalis GA, Angelopoulou MK, Sachanas S, Kontopidou FN, Yiakoumis X, Kokoris SI, Dimitriadou EM, Dimopoulou MN, Moschogiannis M, Korkolopoulou P, Kyrtsonis MC, Siakantaris MP, Papadaki T, Tsaftaridis P, Plata E, Papadaki HE, Vassilakopoulos TP (2013). “Treatment of splenic marginal zone lymphoma with rituximab monotherapy: progress report and comparison with splenectomy”. Oncologist. 18 (2): 190–7. doi:10.1634/theoncologist.2012-0251. PMC 3579603. PMID 23345547.

[pmid27030961-5] Perrone S, D’Elia GM, Annechini G, Ferretti A, Tosti ME, Foà R, Pulsoni A (May 2016). “Splenic marginal zone lymphoma: Prognostic factors, role of watch and wait policy, and other therapeutic approaches in the rituximab era”. Leuk. Res. 44: 53–60. doi:10.1016/j.leukres.2016.03.005. PMID 27030961.

[pmid24057925-6] Olszewski AJ, Ali S (March 2014). “Comparative outcomes of rituximab-based systemic therapy and splenectomy in splenic marginal zone lymphoma”. Ann. Hematol. 93 (3): 449–58. doi:10.1007/s00277-013-1900-4. PMID 24057925.

[pmid23016878-7] 7.0 ^7.1 Else M, Marín-Niebla A, de la Cruz F, Batty P, Ríos E, Dearden CE, Catovsky D, Matutes E (November 2012). “Rituximab, used alone or in combination, is superior to other treatment modalities in splenic marginal zone lymphoma”. Br. J. Haematol. 159 (3): 322–8. doi:10.1111/bjh.12036. PMID 23016878.

[pmid8652403-8] Troussard X, Valensi F, Duchayne E, Garand R, Felman P, Tulliez M, Henry-Amar M, Bryon PA, Flandrin G (June 1996). “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. 93 (3): 731–6. PMID 8652403.

[pmid9359516-9] Bolam S, Orchard J, Oscier D (October 1997). “Fludarabine is effective in the treatment of splenic lymphoma with villous lymphocytes”. Br. J. Haematol. 99 (1): 158–61. PMID 9359516.

[pmid23712547-10] Cervetti G, Galimberti S, Pelosini M, Ghio F, Cecconi N, Petrini M (September 2013). “Significant efficacy of 2-chlorodeoxyadenosine{+/-} rituximab in the treatment of splenic marginal zone lymphoma (SMZL): extended follow-up”. Ann. Oncol. 24 (9): 2434–8. doi:10.1093/annonc/mdt181. PMID 23712547.

[pmid24799461-11] Arcaini L, Vallisa D, Rattotti S, Ferretti VV, Ferreri AJ, Bernuzzi P, Merli M, Varettoni M, Chiappella A, Ambrosetti A, Tucci A, Rusconi C, Visco C, Spina M, Cabras G, Luminari S, Tucci M, Musto P, Ladetto M, Merli F, Stelitano C, d’Arco A, Rigacci L, Levis A, Rossi D, Spedini P, Mancuso S, Marino D, Bruno R, Baldini L, Pulsoni A (July 2014). “Antiviral treatment in patients with indolent B-cell lymphomas associated with HCV infection: a study of the Fondazione Italiana Linfomi”. Ann. Oncol. 25 (7): 1404–10. doi:10.1093/annonc/mdu166. PMID 24799461.

[pmid25285757-12] Rossotti R, Travi G, Pazzi A, Baiguera C, Morra E, Puoti M (January 2015). “Rapid clearance of HCV-related splenic marginal zone lymphoma under an interferon-free, NS3/NS4A inhibitor-based treatment. A case report”. J. Hepatol. 62 (1): 234–7. doi:10.1016/j.jhep.2014.09.031. PMID 25285757.

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