Nodal marginal zone lymphoma
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Synonyms and keywords:: NMZL; Nodal marginal zone B cell lymphoma;  Monocytoid B-cell lymphoma; Nodal monocytoid B-cell lymphoma

# Overview
Nodal marginal zone lymphoma is a rare B-cell neoplasm. Nodal marginal zone lymphoma usually only involves the lymph nodes. Sometimes it can also be found in the bone marrow. A very small number of people with nodal marginal zone lymphoma will also have hepatitis C infection (HCV). On microscopic histopathological analysis, centrocytes, centroblasts, plasma cells, and immunoblasts are characteristic findings of nodal marginal zone lymphoma. The incidence of nodal marginal zone lymphoma increases with age; the median age at diagnosis is 60 years. Females are more commonly affected with nodal marginal zone lymphoma than males. Nodal marginal zone lymphoma is often slow growing (indolent). However, it can transform into a more aggressive large cell lymphoma. Nodal marginal zone lymphomas behave slightly more aggressively than MALT lymphomas. Symptoms of the nodal marginal zone lymphoma include fever, weight loss, night sweats, and painless swelling in the neck, axilla, groin, thorax, and abdomen. Common physical examination findings of nodal marginal zone lymphoma include fever, peripheral lymphadenopathy, and central lymphadenopathy. Lymph node or bone marrow biopsy is diagnostic of nodal marginal zone lymphoma. The predominant therapy for nodal marginal zone lymphoma is chemotherapy. Adjunctive radiotherapy, stem cell transplant, and biological therapy may be required. The optimal therapy for nodal marginal zone lymphoma depends on the clinical presentation.

# Pathophysiology
- Nodal marginal zone lymphoma is a rare B-cell neoplasm.
- It arises from marginal zone B cells.
- Nodal marginal zone lymphoma usually only involves the lymph nodes. Sometimes it can also be found in the bone marrow.
- Mutations in oncogenes and tumor suppressor genes is considered to be involved in pathogenesis of nodal marginal zone lymphoma.
- Gene sequencing shows mutations in KMT2D, PTTPRD, NOTCH2, KLF2.

## Associated Conditions
A very small number of people with nodal marginal zone lymphoma will also have
- Hepatitis C infection (HCV)
- Autoimmune disorders

## Microscopic Pathology
On microscopic histopathological analysis of marginal zone lymphoma.,the following features are seen:
- Centrocytes
- Centroblasts
- Plasma cells
- Immunoblasts

# Causes
There are no established causes for nodal marginal zone lymphoma.

# Differentiating Nodal Marginal Zone Lymphoma from other Diseases
Nodal marginal zone lymphoma must be differentiated from other diseases such as:
- Chronic lymphocytic leukemia
- Splenic marginal zone lymphoma
- Mantle cell lymphoma
- Diffuse large B cell lymphoma (DLBCL)
- Lymphoplasmacytic lymphoma
- Waldenstroms macroglobulinemia

# Epidemiology and Demographics

## Age
The incidence of nodal marginal zone lymphoma increases with age; the median age at diagnosis is 60 years.

## Gender
Females are more commonly affected with nodal marginal zone lymphoma than males.

# Risk Factors
There are no established risk factors for nodal marginal zone lymphoma.

# Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for nodal marginal zone lymphoma.

# Natural History, Complications and Prognosis
- Nodal marginal zone lymphoma is often slow growing (indolent)
- NMZL can transform into a more aggressive large cell lymphoma.
- Nodal marginal zone lymphomas behave slightly more aggressively than MALT lymphomas.
- Low grade marginal zone lymphoma has a sluggish course and excellent response to early therapy.
- Prognosis depends on the primary organ of origin.
- Those lymphomas which are positive for (11;18) translocation have the worst response to therapy.

# Diagnosis

## Staging
Staging for nodal marginal zone lymphoma is provided in the following table:

## Symptoms
Symptoms of the nodal marginal zone lymphoma include:
- Fever
- Weight loss
- Night sweats
- Painless swelling in the neck, axilla, groin, thorax, and abdomen

## Physical Examination

### Vitals
- Fever is often present

### HEENT
- Cervical lymphadenopathy

### Thorax
- Thoracic masses suggestive of central lymphadenopathy

### Abdomen
- Abdominal masses suggestive of central lymphadenopathy

### Extremities
- Peripheral lymphadenopathy

## Laboratory Findings
Laboratory tests for nodal marginal zone lymphoma include:
- Complete blood count (CBC)
- Blood chemistry studies
- Cytogenetic analysis
- Flow cytometry
- Immunohistochemistry
- Immunophenotyping:
- Positive: CD19, CD79a, bcl2, and  CD22
- Negative: CD5, CD10, CD23, CD25, bcl10, and cyclin D1

### Biopsy
Lymph node or bone marrow biopsy is diagnostic of nodal marginal zone lymphoma.

### CT
CT scan may be helpful in the diagnosis of nodal marginal zone lymphoma.

### MRI
MRI scan may be helpful in the diagnosis of nodal marginal zone lymphoma.

### Other Imaging Findings
PET scan may be helpful in the diagnosis of nodal marginal zone lymphoma.

# Treatment

## Medical Therapy