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Marginal Zone Lymphoma

Indolent B-cell lymphomas of MALT, splenic, and nodal origin

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Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →

What is Marginal Zone Lymphoma?

Marginal zone lymphomas account for approximately 8-10% of non-Hodgkin lymphomas with three main subtypes: extranodal MZL of mucosa-associated lymphoid tissue (MALT lymphoma, 50-70% of MZLs), splenic MZL (20%), and nodal MZL (10%). Pathogenesis involves chronic antigen stimulation from infection (Helicobacter pylori in gastric MALT, Borrelia burgdorferi in cutaneous MALT, Chlamydia psittaci in ocular adnexal MALT, hepatitis C in splenic MZL) or autoimmune disorders (Sjögren's, Hashimoto's), leading to monoclonal B-cell expansion with characteristic chromosomal translocations.

Clinical features depend on subtype: extranodal MALT presents with site-specific symptoms (gastric pain and dyspepsia, salivary gland swelling, ocular adnexal mass, cutaneous lesions, pulmonary nodules), splenic MZL with splenomegaly, lymphocytosis, autoimmune cytopenias, and constitutional symptoms, and nodal MZL with painless lymphadenopathy. Diagnosis requires tissue biopsy with immunohistochemistry (CD20+, CD5-, CD10-, BCL2+, IgM+), flow cytometry, and molecular studies for translocations (t(11;18), t(1;14), t(14;18)).

Staging includes CT or PET-CT, bone marrow biopsy, complete blood count, comprehensive metabolic panel, lactate dehydrogenase, beta-2-microglobulin, and serum protein electrophoresis. Helicobacter pylori testing for gastric MALT. Treatment varies by subtype: localized gastric MALT often responds to H. pylori eradication alone (60-80% remission), other localized cases benefit from radiation or surgery, advanced stage disease treated with rituximab monotherapy or with chemotherapy (R-bendamustine, R-CHOP), and watchful waiting for asymptomatic indolent disease. Prognosis is excellent with 10-year overall survival 80-90%.

Symptoms

Painless lymphadenopathy (nodal MZL)
Splenomegaly (splenic MZL)
Gastric pain and dyspepsia (gastric MALT)
Salivary gland swelling (parotid MALT)
Ocular adnexal mass (orbital MALT)
Cutaneous lesions (cutaneous MALT)
Pulmonary nodules (BALT)
Lacrimal gland enlargement
Mediastinal mass
Thyroid enlargement (Hashimoto-associated)
Constitutional symptoms (B-symptoms): fever, night sweats, weight loss
Fatigue
Anemia
Thrombocytopenia
Autoimmune cytopenias
Lymphocytosis (splenic MZL)
Cryoglobulinemia (HCV-associated splenic MZL)
Skin lesions (annular plaques, papules)
Hepatomegaly
Bone marrow involvement

Risk Factors

Helicobacter pylori infection (gastric MALT)
Borrelia burgdorferi infection (cutaneous MALT)
Chlamydia psittaci infection (ocular adnexal MALT)
Hepatitis C virus infection (splenic MZL)
Sjögren's syndrome (parotid MALT)
Hashimoto's thyroiditis (thyroid MALT)
Autoimmune disorders (rheumatoid arthritis, lupus)
Chronic gastritis
Immunosuppression
HIV infection
Older age (median 60 years)
Female gender (slight predominance)
Caucasian ethnicity
Family history of lymphoma
Prior chemotherapy or radiation
Solid organ transplantation
Celiac disease
Inflammatory bowel disease
Smoking
Pesticide exposure

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent lymphadenopathy
  • Splenomegaly with constitutional symptoms
  • Refractory gastric symptoms despite treatment
  • Recurrent dyspepsia with weight loss
  • Salivary gland enlargement
  • Ocular adnexal mass
  • Persistent skin lesions
  • Lymphocytosis on routine blood work
  • Autoimmune cytopenias
  • Hepatitis C with new symptoms
  • Sjögren's syndrome with new mass
  • Hashimoto's thyroiditis with thyroid nodule
  • Family history of lymphoma with concerning symptoms
  • Treatment-related complications

Treatment Methods

01
Comprehensive evaluation by hematologist-oncologist
02
Detailed history including infections, autoimmune diseases, family history
03
Physical examination with lymph node assessment, splenomegaly, organ involvement
04
Tissue biopsy with immunohistochemistry (CD20+, CD5-, CD10-, BCL2+, IgM+)
05
Flow cytometry for clonality and immunophenotyping
06
Molecular studies for translocations (t(11;18), t(1;14), t(14;18))
07
Helicobacter pylori testing for gastric MALT
08
Hepatitis C virus testing for splenic MZL
09
Borrelia burgdorferi testing for cutaneous MALT
10
Chlamydia psittaci testing for ocular adnexal MALT
11
Bone marrow biopsy and aspirate
12
CT or PET-CT for staging
13
Endoscopy with biopsy for gastric MALT
14
Complete blood count, comprehensive metabolic panel, LDH, beta-2-microglobulin
15
Serum protein electrophoresis with immunofixation
16
Cryoglobulin testing if indicated
17
Helicobacter pylori eradication for localized gastric MALT (60-80% remission)
18
Radiation therapy for localized non-gastric extranodal MALT
19
Surgical excision for selected localized lesions
20
Watchful waiting for asymptomatic indolent disease
21
Rituximab monotherapy for symptomatic indolent disease
22
R-bendamustine for advanced stage disease
23
R-CHOP for transformation or aggressive features
24
Splenectomy for splenic MZL with cytopenias or splenomegaly
25
Antiviral therapy for HCV-associated splenic MZL
26
Antibiotic therapy for infection-associated MALT (clarithromycin, doxycycline)
27
Treatment of underlying autoimmune disease
28
Stem cell transplantation for transformed or refractory disease
29
Long-term surveillance with imaging and laboratory monitoring
30
Multidisciplinary care including hematology, gastroenterology, ophthalmology
31
Supportive care for symptoms and treatment side effects
32
Patient education on prognosis and surveillance

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.