Overview
Marginal zone lymphoma (MZL) is a type of slow-growing (indolent) blood cancer that develops from a specific kind of white blood cell called a B-lymphocyte. These B-cells normally live in an area of the lymph nodes and spleen called the marginal zone, which is part of your immune system. When these cells grow out of control, they form tumors in lymph nodes, the spleen, or other organs. There are three main subtypes of marginal zone lymphoma. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is the most common type and often develops in the stomach, lungs, eyes, or skin. Splenic marginal zone lymphoma starts in the spleen and can affect the blood and bone marrow. Nodal marginal zone lymphoma begins in the lymph nodes. Symptoms vary depending on where the lymphoma develops but may include painless swollen lymph nodes, fatigue, unexplained weight loss, night sweats, and abdominal discomfort if the spleen is enlarged. Many patients have no symptoms at all, and the disease is found during routine blood tests or exams. Treatment depends on the subtype and stage and can range from watchful waiting (active surveillance) to antibiotics (for stomach MALT linked to H. pylori infection), radiation therapy, immunotherapy with rituximab, chemotherapy, or combinations of these. Because MZL tends to grow slowly, many patients live for many years with appropriate management.
Key symptoms:
Painless swollen lymph nodesFatigue and tirednessUnexplained weight lossNight sweatsFever without infectionEnlarged spleen causing fullness or pain in the upper left abdomenStomach pain or indigestion (for gastric MALT type)Nausea or vomitingLow red blood cell count (anemia)Low platelet count causing easy bruising or bleedingSkin lumps or rashes (for skin involvement)Eye redness, swelling, or blurred vision (for eye involvement)Feeling full quickly when eatingRecurrent infections
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
1 eventInternational Extranodal Lymphoma Study Group (IELSG)
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
1 availableBRUKINSA
BRUKINSA is indicated for the treatment of adult patients with relapsed or refractory marginal zone lymphoma (MZL) who have received at least one anti–CD20-based regimen.
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Marginal zone lymphoma.
Community
No community posts yet. Be the first to share your experience with Marginal zone lymphoma.
Start the conversation →Latest news about Marginal zone lymphoma
No recent news articles for Marginal zone lymphoma.
Follow this condition to be notified when news becomes available.
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What subtype of marginal zone lymphoma do I have, and what stage is it?,Do I need treatment right away, or is watchful waiting appropriate for me?,If I have gastric MALT lymphoma, has H. pylori testing been done?,What treatment options do you recommend, and what are the expected side effects?,What is the chance that my lymphoma could transform into a more aggressive type?,How often will I need follow-up visits and scans?,Are there any clinical trials available that might be right for me?
Common questions about Marginal zone lymphoma
What is Marginal zone lymphoma?
Marginal zone lymphoma (MZL) is a type of slow-growing (indolent) blood cancer that develops from a specific kind of white blood cell called a B-lymphocyte. These B-cells normally live in an area of the lymph nodes and spleen called the marginal zone, which is part of your immune system. When these cells grow out of control, they form tumors in lymph nodes, the spleen, or other organs. There are three main subtypes of marginal zone lymphoma. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is the most common type and often develops in the stomach, lungs,
How is Marginal zone lymphoma inherited?
Marginal zone lymphoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Marginal zone lymphoma typically begin?
Typical onset of Marginal zone lymphoma is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Marginal zone lymphoma?
Yes — 1 recruiting clinical trial is currently listed for Marginal zone lymphoma on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Marginal zone lymphoma?
25 specialists and care centers treating Marginal zone lymphoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.