DelveInsight’s ’Marginal Zone Lymphoma-Market Insights, Epidemiology, and Market Forecast–2030’ report deliver an in-depth understanding of the MZL, historical and forecasted epidemiology as well as the MZL market trends in the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom) and Japan.
The Marginal Zone Lymphoma market report provides current treatment practices, emerging drugs, and market share of the individual therapies, current and forecasted 7MM MZL market size from 2017 to 2030. The Report also covers current MZL treatment practice, market drivers, market barriers, SWOT analysis, reimbursement, and market access, and unmet medical needs to curate the best of the opportunities and assesses the underlying potential of the market.
Geography Covered

  • The United States
  • EU5 (Germany, France, Italy, Spain, and the United Kingdom)
  • Japan



Study Period: 2017–2030
Marginal Zone Lymphoma Disease Understanding and Treatment Algorithm
Marginal Zone Lymphoma Overview
Marginal zone B-cell lymphoma or Marginal Zone Lymphoma (MZL) is a group of indolent (slow-growing) NHL B-cell lymphomas, which account for approximately 5_17% of all NHL cases. In the World Health Organization classification, there are three different MZL entities with specific diagnostic criteria, behavior, and therapeutic implications: the extranodal MZL of mucosa-associated lymphoid tissue (MALT) type (MALT lymphoma), the splenic MZL (SMZL), and the nodal MZL (NMZL).
Extranodal marginal zone B-cell lymphoma, also known as mucosa-associated lymphoid tissue (MALT) lymphoma: This is the most common type of marginal zone lymphoma. It starts in places other than the lymph nodes (extranodal). Nodal MZL (sometimes called monocytoid B-cell lymphoma) occurs within the lymph nodes and accounts for about 10% of all MZL cases. In most cases, it is not known what causes nodal MZL. It is more common in people who have been infected with the hepatitis C virus and some autoimmune conditions than others. Splenic MZL occurs most often in the spleen and blood. It has been associated with hepatitis C virus (HCV) infection. It is a low-grade B-cell non-Hodgkin’s lymphoma characterized by massive splenomegaly, moderate lymphocytosis with or without villous lymphocytes, rare involvement of peripheral lymph nodes, and indolent clinical course.


Marginal Zone Lymphoma Diagnosis
To make a diagnosis, the doctor would need to stage the disease. Staging is also how the doctor decides the right treatment. It involves evaluating the location and size of the tumors and determining whether cancer has spread to other parts of the body. The diagnosis can be established based on the endoscopic appearance. The endoscopist must take specimens from the antrum and the corpus for the urease test. If the examined segments are unsuspicious, a biopsy must be performed from each quadrant of the fundus, the corpus, and the antrum and ten biopsies if they are suspicious, to request a histological workup.
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Marginal Zone Lymphoma Treatment
Treatment selection for a patient with Marginal Zone Lymphoma (MZL) depends on the type, stage, and location of the disease. Since gastric MALT lymphoma is often the result of an infection with H. pylori, the initial treatment is antibiotic therapy, usually combined with proton pump inhibitors (PPIs). The most commonly used regimen is triple therapy: a proton pump inhibitor (omeprazole) in association with amoxicillin and clarithromycin. Metronidazole can be substituted for amoxicillin in penicillin-allergic individuals. If the lymphoma relapses (disease returns after treatment) or becomes refractory (disease does not respond to treatment) after antibiotic therapy, there are many additional treatment options available, including rituximab, radiation therapy, and surgery. For SMZL, the recognized therapeutic options are splenectomy, chemotherapy (ChT), rituximab alone, or rituximab plus chemotherapy. Nodal MZL usually responds well to chemotherapy. It may be given as a single drug or a combination of drugs and is often given with a targeted therapy drug. Single drugs that may be used for nodal MZL are fludarabine (Fludara), bendamustine (Treanda). Combinations of chemotherapy drugs that may be used are: CHOP,R-CHOP,CVP,R-CVP and BR.
Marginal Zone Lymphoma Epidemiology
The disease epidemiology covered in the report provides historical as well as forecasted epidemiology segmented by Total incident cases of Non-Hodgkin lymphoma, Total incident cases of Marginal Zone Lymphoma, Gender-specific incident cases of MZL, Subtype-specific incident cases of MZL and Stage-specific incident cases of MZL scenario of MZL in the 7MM covering the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom) and Japan from 2017–2030.

Key Findings

  • The total incident population of Non-Hodgkin lymphoma (NHL) in the 7MM was anticipated to be 166,967 in 2017.
  • The total incident population of MZL in the 7MM was anticipated to be 21,284 in 2017 and is expected to rise by 2030.
  • According to the estimates, the highest market size of Marginal Zone Lymphoma (MZL) was found in the United States, followed by Japan
  • The epidemiological model of DelveInsight estimates a higher proportion of MZL cases was found in stage III as compared to other stages in the United States. In 2017, stage I, stage II, and stage III MZL incident cases were 1,416, 465, and 5,307 respectively, in the US.


Marginal Zone Lymphoma Epidemiology
The epidemiology segment also provides the MZL epidemiology data and findings across the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom) and Japan.
Marginal Zone Lymphoma Drug Chapters
The drug chapter segment of the MZL report encloses the detailed analysis of MZL marketed drugs, mid-phase, and late-stage pipeline drugs. It also helps to understand the MZL clinical trial details, expressive pharmacological action, agreements and collaborations, approval, and patent details of each included drug and the latest news and press releases.
Marginal Zone Lymphoma Marketed Drugs
Revlimid (lenalidomide) in combination with rituximab: Celgene Corporation, Bristol-Myers Squibb
Revlimid is an immune-modulating therapy with proven anti-myeloma e_ects. It is an oral therapy that was shown to work in three ways in animal models and in vitro, it helps the immune system recognize and destroy myeloma cells. It has the ability to targets and kills myeloma cells and helps prevent new myeloma cell growth by starving them of blood. In May 2019, FDA approved lenalidomide Revlimid in combination with a rituximab product for previously treated FL and previously-treated MZL.
Products detail in the report?
Imbruvica (ibrutinib): AbbVie, Janssen Biotech
Ibrutinib is a small-molecule inhibitor of BTK. The drug forms a covalent bond with a cysteine residue in the BTK active site, leading to inhibition of BTK enzymatic activity. BTK is a signaling molecule of the B-cell antigen receptor (BCR) and cytokine receptor pathways. BTK’s role in signaling through the B-cell surface receptors results in the activation of pathways necessary for B-cell trafficking, chemotaxis, and adhesion. Nonclinical studies show that ibrutinib inhibits malignant B-cell proliferation and survival in vivo as well as cell migration and substrate adhesion in vitro.
The recommended dose of Imbruvica for MCL and MZL is 560 mg orally once daily until disease progression or unacceptable toxicity.
Products detail in the report?
Marginal Zone Lymphoma Emerging Drugs
Umbralisib (TGR-1202): TG Therapeutics
Umbralisib (TGR-1202) is an orally administered, investigational dual inhibitor of PI3K delta and CK1 epsilon, which is administered orally once daily. The phosphoinositide-3-kinases (PI3Ks) are a family of enzymes involved in many important cellular functions, including cell proliferation and survival, cell differentiation, intracellular trafficking, and immunity. There are four isoforms of PI3K (alpha, beta, delta, and gamma), of which the delta isoform is highly expressed in hematopoietic cells. Dysregulation of the PI3K pathway is among one of the most commonly mutated pathways across all of cancer biology.
Currently, TG Therapeutics is investigating the drug in Phase II/III clinical in a combination of ublituximab, with or without Bendamustine, and TGR-1202 alone in patients with previously treated non-Hodgkin’s lymphoma (NHL).
Products detail in the report?
Aliqopa (Copanlisib): Bayer
Aliqopa (Copanlisib) is an intravenously administered, small molecule with inhibitory activity against all four isoforms including the PI3K-alpha and PI3K-delta isoforms expressed in malignant B cells. The PI3K pathway is involved in cell growth, survival, and metabolism, and its dysregulation plays an important role in the development of lymphoma. The drug is also the only PI3K inhibitor administered intravenously on an intermittent schedule: on days 1, 8, and 15 of a 28-day treatment cycle. Treatment should be continued until disease progression or unacceptable toxicity. Copanlisib is currently approved in the US and Taiwan under the brand name Aliqopa for relapsed follicular lymphoma (FL). In September 2017, the US FDA granted accelerated approval to the drug for the treatment of adult patients with relapsed follicular lymphoma who have received at least two prior systemic therapies. The drug is currently in phase III for MZL.
Products detail in the report?
Ublituximab: TG Therapeutics
Ublituximab (TG-1101) is an investigational glycoengineered monoclonal antibody that targets a unique epitope on CD20-expressing B-cells. When ublituximab binds to the B-cell, it triggers a series of immunological reactions (including antibody-dependent cellular cytotoxicity [ADCC] and complement-dependent cytotoxicity [CDC]), leading to the destruction of the cell. Additionally, ublituximab is uniquely designed, to lack certain sugar molecules normally expressed on the antibody. Removal of these sugar molecules, a process called glycoengineering, has been shown to enhance the potency of ublituximab, especially the ADCC activity.
Products detail in the report?
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Marginal Zone Lymphoma Market Outlook
A Marginal zone lymphomas (MZL) involves immune cells known as B-cells and accounts for between 5% and 17% of all non-Hodgkin lymphomas (NHL). The three main subtypes of MZL are extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), nodal MZL, and splenic MZL. Of these, MALT lymphoma is the most common. The symptoms that may be caused by MZL depend on the type and location of the MZL. MALT lymphoma commonly involves the stomach, and patients may report abdominal pain, heartburn, and indigestion. MALT lymphoma may also develop in other areas of the body, such as the area around the eye, the salivary glands, or the lungs. Splenic MZL may be detected because of the enlargement of the spleen or abnormal blood cell counts. These types of lymphoma tend to be indolent (slow-growing).
Due to the slow nature of progression, most patients tend to present before developing significant complications. In the rare cases of life or organ-threatening MZL, high-dose corticosteroids (for example, dexamethasone 40 mg daily) can be used to temporize matters before starting more definitive (Immuno) chemotherapy or radiotherapy. Treatment of indolent lymphoma should be individualized according to the disease subtype, presentation, comorbid conditions, and patient preferences. When possible, the goal of therapy is to cure the disease and/or prolong survival. In all cases, the goal should be to improve quality of life; care must be taken to avoid overtreatment.
There are several different treatment approaches for SMZL, including single-agent rituximab, splenectomy, and traditional chemotherapy. For localized disease, local therapy is recommended such as triple therapy for H. pylori in gastric extranodal MZL, splenectomy for splenic MZL, and radiotherapy for nodal MZL. Also, most patients with nodal MZL present with advanced-stage disease and are not likely to achieve a cure, even with aggressive chemotherapy regimens. For a disseminated disease with a low tumor burden, a watch and wait or single-agent rituximab can be used. However, for symptomatic disease, a similar approach to follicular lymphoma (FL) can be used with chemoimmunotherapy approaches such as bendamustine and rituximab.
Patients with the relapsing disease after at least one CD20-based therapy have several recently approved chemotherapy-free options including B-cell receptor inhibitors such as ibrutinib (approved specifically in MZL) and immunomodulatory agents, such as lenalidomide and rituximab (FDA approved in MZL and FL). Phosphoinositide 3-kinase (PI3K) inhibitors have shown excellent activity in iNHL, specifically in MZL, with breakthrough designation status for copanlisib and umbralisib, allowing off label use of this class of agents in clinical practice.
Patients with early-stage MALT lymphoma with no underlying infectious etiology, or those for whom antibiotic therapy has failed, can be effectively managed with external beam radiotherapy. Rarely, marginal zone lymphoma can transform into a more aggressive lymphoma, typically DLBCL. These patients require aggressive therapy and may benefit from autologous stem cell transplantation. Although the aggressive subtype may resolve completely, patients are often left with a persistent low-grade lymphoma.

Key Findings

  • The market size of Marginal Zone Lymphoma (MZL) in the 7MM was estimated to be USD 427.16 million in 2017
  • Among the EU-5 countries, the UK has the highest and same market size of USD 43.85 million. The lowest market size was estimated in Spain with USD 10.65 million in 2017
  • Umbralisib (TGR-1202), Duvelisib, Ublituximab, Aliqopa (copanlisib), and Calquence (Acalabrutinib) are pipeline molecules that are expected to launch as second-line therapy in the forecast period (2020_2030)
  • The market size of first-line therapies in the 7MM was estimated to be USD 234.24 million in 2017.
  • The market size of second-line therapies was observed to be USD 162.33 million in the 7MM, in 2017, and the market size of third-line therapies was observed to be USD 30.59 million in the 7MM, in 2017.




The United States Market Outlook
This section provides the total MZL market size and; market size by line of therapies in the United States.
EU-5 Market Outlook
The total MZL market size and market size by line of therapies in Germany, France, Italy, Spain, and the United Kingdom are provided in this section.
Japan Market Outlook
The total MZL market size and market size by line of therapies in Japan are provided.
Marginal Zone Lymphoma Drugs Uptake
This section focusses on the rate of uptake of the potential drugs recently launched in the MZL market or expected to get launched in the market during the study period 2017–2030. The analysis covers the MZL market uptake by drugs; patient uptake by therapies; and sales of each drug.
This helps in understanding the drugs with the most rapid uptake, reasons behind the maximal use of new drugs, and allows the comparison of the drugs based on market share and size which again will be useful in investigating factors important in market uptake and in making financial and regulatory decisions.
Marginal Zone Lymphoma Development Activities
The report provides insights into different therapeutic candidates in phase II, and phase III stage. It also analyzes key players involved in developing targeted therapeutics.
Pipeline Development Activities
The report covers the detailed information of collaborations, acquisition, and merger, licensing, and patent details for Marginal Zone Lymphoma emerging therapies.
Reimbursement Scenario in Marginal Zone Lymphoma
Approaching reimbursement proactively can have a positive impact both during the late stages of product development and well after product launch. In the report, we consider reimbursement to identify economically attractive indications and market opportunities. When working with finite resources, the ability to select the markets with the fewest reimbursement barriers can be a critical business and price strategy.
Competitive Intelligence Analysis
We perform competitive and market Intelligence analysis of the Marginal Zone Lymphoma market by using various competitive intelligence tools that include–SWOT analysis, PESTLE analysis, Porter’s five forces, BCG Matrix, Market entry strategies, etc. The inclusion of the analysis entirely depends upon the data availability.
Scope of the Report

  • The report covers the descriptive overview of MZL, explaining its causes, symptoms, pathophysiology, genetic basis, and currently available therapies.
  • Comprehensive insight has been provided into the MZL epidemiology and treatment.
  • Additionally, an all-inclusive account of both the current and emerging therapies for MZL is provided, along with the assessment of new therapies, which will have an impact on the current treatment landscape.
  • A detailed review of the MZL market; historical and forecasted is included in the report, covering the 7MM drug outreach.
  • The report provides an edge while developing business strategies, by understanding trends shaping and driving the 7MM MZL market.



Report Highlights

  • The robust pipeline with novel MOA and oral ROA, increasing prevalence, effectiveness of drugs as both mono and combination therapy will positively drive the MZL market.
  • The companies and academics are working to assess challenges and seek opportunities that could influence MZL R&D. The therapies under development are focused on novel approaches to treat/improve the disease condition.
  • Major players are involved in developing therapies for MZL. Launch of emerging therapies will significantly impact the MZL market.
  • Our in-depth analysis of the pipeline assets across different stages of development (phase III and phase II), different emerging trends and comparative analysis of pipeline products with detailed clinical profiles, key cross-competition, launch date along with product development activities will support the clients in the decision-making process regarding their therapeutic portfolio by identifying the overall scenario of the research and development activities.


Marginal Zone Lymphoma Report Insights

  • Patient Population
  • Therapeutic Approaches
  • MZL Pipeline Analysis
  • MZL Market Size and Trends
  • Market Opportunities
  • Impact of upcoming Therapies


Marginal Zone Lymphoma Report Key Strengths

  • 11 Years Forecast
  • 7MM Coverage
  • MZL Epidemiology Segmentation
  • Key Cross Competition
  • Highly Analyzed Market
  • Drugs Uptake



Marginal Zone Lymphoma Report Assessment

  • Current Treatment Practices
  • Unmet Needs
  • Pipeline Product Profiles
  • Market Attractiveness
  • Market Drivers and Barriers
  • SWOT analysis


Key Questions
Market Insights:

  • What was the MZL market share (%) distribution in 2017 and how it would look like in 2030?
  • What would be the MZL total market size as well as market size by therapies across the 7MM during the forecast period (2020–2030)?
  • What are the key findings pertaining to the market across the 7MM and which country will have the largest MZL market size during the forecast period (2020–2030)?
  • At what CAGR, the MZL market is expected to grow at the 7MM level during the forecast period (2020–2030)?
  • What would be the MZL market outlook across the 7MM during the forecast period (2020–2030)?
  • What would be the MZL market growth till 2030 and what will be the resultant market size in the year 2030?
  • How would the market drivers, barriers, and future opportunities affect the market dynamics and subsequent analysis of the associated trends?


Epidemiology Insights:

  • What is the disease risk, burden, and unmet needs of MZL?
  • What is the historical MZL patient pool in the United States, EU5 (Germany, France, Italy, Spain, and the UK) and Japan?
  • What would be the forecasted patient pool of MZL at the 7MM level?
  • What will be the growth opportunities across the 7MM with respect to the patient population pertaining to MZL?
  • Out of the above-mentioned countries, which country would have the highest prevalent population of MZL during the forecast period (2020–2030)?
  • At what CAGR the population is expected to grow across the 7MM during the forecast period (2020–2030)?


Current Treatment Scenario, Marketed Drugs, and Emerging Therapies:

  • What are the current options for the treatment of MZL along with the approved therapy?
  • What are the current treatment guidelines for the treatment of MZL in the US and Europe?
  • What are the MZL marketed drugs and their MOA, regulatory milestones, product development activities, advantages, disadvantages, safety, and efficacy, etc.?
  • How many companies are developing therapies for the treatment of MZL?
  • How many emerging therapies are in the mid-stage and late stages of development for the treatment of MZL?
  • What are the key collaborations (Industry–Industry, Industry-Academia), Mergers and acquisitions, licensing activities related to the MZL therapies?
  • What are the recent novel therapies, targets, mechanisms of action, and technologies developed to overcome the limitation of existing therapies?
  • What are the clinical studies going on for MZL and their status?
  • What are the key designations that have been granted for the emerging therapies for MZL?
  • What are the 7MM historical and forecasted market of MZL?



Reasons To Buy

  • The report will help in developing business strategies by understanding trends shaping and driving the MZL.
  • To understand the future market competition in the MZL market and Insightful review of the key market drivers and barriers.
  • Organize sales and marketing efforts by identifying the best opportunities for MZL in the US, Europe (Germany, Spain, Italy, France, and the United Kingdom) and Japan.
  • Identification of strong upcoming players in the market will help in devising strategies that will help in getting ahead of competitors.
  • Organize sales and marketing efforts by identifying the best opportunities for the MZL market.
  • To understand the future market competition in the MZL market.