Marginal zone lymphoma

Old, new, targeted, and epigenetic therapies

Monika Joshi, Hassan Sheikh, Kamal Abbi, Kamal Sharma, Mark Tulchinsky, Elliot Epner, Sarah Long

Research output: Contribution to journalReview article

18 Citations (Scopus)

Abstract

Marginal zone lymphoma (MZL) is an indolent B-cell lymphoma arising from marginal zone B-cells present in lymph nodes and extranodal tissues. MZL comprises 5–17% of all non-Hodgkin’s lymphomas in adults. The World Health Organization categorizes MZL into three distinct types based on their site of impact: (1) splenic marginal zone lymphoma (SMZL); (2) nodal marginal zone lymphoma (NMZL); (3) extranodal mucosa-associated lymphoid tissue (MALT) lymphoma, which can be subdivided into gastric and nongastric. The subgroups of MZL share some common features but are different in their biology and behavior. Owing to the rarity of MZL there are few randomized trials available comparing various treatment options and therefore treatment is controversial, lacking standard guidelines. Treatment should be patient tailored and can range from a ‘watchful waiting’ approach for asymptomatic patients without cytopenias to surgery or localized radiation therapy. Rituximab in combination with chemotherapy has resulted in longer failure-free survival than chemotherapy alone in patients with SMZL. Helicobacter pylori positive gastric MALT shows a good response rate to triple antibiotic therapy. Newer therapies such as bendamustine, everolimus, lenalidomide, vorinostat and phosphoinositide 3-kinase inhibitors are in clinical trials for patients with relapsed or refractory MZL and have shown promising results. We are presently conducting clinical trials testing the efficacy of the epigenetic activity of cladribine as a hypomethylating agent in combination with the histone deacetylase inhibitor (HDACi) vorinostat and rituximab in patients with MZL. Further studies with the newer agents should be done both in newly diagnosed or relapsed/refractory MZL to streamline the care and to avoid the use of toxic chemotherapies as initial treatment.

Original languageEnglish (US)
Pages (from-to)275-290
Number of pages16
JournalTherapeutic Advances in Hematology
Volume3
Issue number5
DOIs
StatePublished - Jan 1 2012

Fingerprint

Epigenomics
Lymphoma
Therapeutics
Marginal Zone B-Cell Lymphoma
Clinical Trials
Cladribine
Watchful Waiting
Drug Therapy
Histone Deacetylase Inhibitors
1-Phosphatidylinositol 4-Kinase
Poisons
B-Cell Lymphoma
Lymphoid Tissue
Gastric Mucosa
Combination Drug Therapy
Helicobacter pylori
Non-Hodgkin's Lymphoma
Stomach
Radiotherapy
Lymph Nodes

All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

Joshi, Monika ; Sheikh, Hassan ; Abbi, Kamal ; Sharma, Kamal ; Tulchinsky, Mark ; Epner, Elliot ; Long, Sarah. / Marginal zone lymphoma : Old, new, targeted, and epigenetic therapies. In: Therapeutic Advances in Hematology. 2012 ; Vol. 3, No. 5. pp. 275-290.
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abstract = "Marginal zone lymphoma (MZL) is an indolent B-cell lymphoma arising from marginal zone B-cells present in lymph nodes and extranodal tissues. MZL comprises 5–17{\%} of all non-Hodgkin’s lymphomas in adults. The World Health Organization categorizes MZL into three distinct types based on their site of impact: (1) splenic marginal zone lymphoma (SMZL); (2) nodal marginal zone lymphoma (NMZL); (3) extranodal mucosa-associated lymphoid tissue (MALT) lymphoma, which can be subdivided into gastric and nongastric. The subgroups of MZL share some common features but are different in their biology and behavior. Owing to the rarity of MZL there are few randomized trials available comparing various treatment options and therefore treatment is controversial, lacking standard guidelines. Treatment should be patient tailored and can range from a ‘watchful waiting’ approach for asymptomatic patients without cytopenias to surgery or localized radiation therapy. Rituximab in combination with chemotherapy has resulted in longer failure-free survival than chemotherapy alone in patients with SMZL. Helicobacter pylori positive gastric MALT shows a good response rate to triple antibiotic therapy. Newer therapies such as bendamustine, everolimus, lenalidomide, vorinostat and phosphoinositide 3-kinase inhibitors are in clinical trials for patients with relapsed or refractory MZL and have shown promising results. We are presently conducting clinical trials testing the efficacy of the epigenetic activity of cladribine as a hypomethylating agent in combination with the histone deacetylase inhibitor (HDACi) vorinostat and rituximab in patients with MZL. Further studies with the newer agents should be done both in newly diagnosed or relapsed/refractory MZL to streamline the care and to avoid the use of toxic chemotherapies as initial treatment.",
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Marginal zone lymphoma : Old, new, targeted, and epigenetic therapies. / Joshi, Monika; Sheikh, Hassan; Abbi, Kamal; Sharma, Kamal; Tulchinsky, Mark; Epner, Elliot; Long, Sarah.

In: Therapeutic Advances in Hematology, Vol. 3, No. 5, 01.01.2012, p. 275-290.

Research output: Contribution to journalReview article

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T1 - Marginal zone lymphoma

T2 - Old, new, targeted, and epigenetic therapies

AU - Joshi, Monika

AU - Sheikh, Hassan

AU - Abbi, Kamal

AU - Sharma, Kamal

AU - Tulchinsky, Mark

AU - Epner, Elliot

AU - Long, Sarah

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