Nodal marginal zone lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

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

Pathophysiology

Associated Conditions

A very small number of people with nodal marginal zone lymphoma will also have hepatitis C infection (HCV).

Causes

There are no established causes for nodal marginal zone lymphoma.

Differentiating type page name here 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)

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). However, it can change (transform) into a more aggressive large cell lymphoma. Nodal marginal zone lymphomas behave slightly more aggressively than MALT lymphomas.

Diagnosis

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

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

Treatment of nodal marginal zone lymphoma

Therapy	Description
Watchful waiting	May not need treatment right away unless they have symptoms.
Chemotherapy	Nodal marginal zone lymphoma is usually sensitive to chemotherapy. Monotherapy: Chlorambucil OR Fludarabine Combination therapy : CVP – Cyclophosphamide AND Vincristine AND Prednisone CHOP – Cyclophosphamide AND Doxorubicin AND Vincristine AND Prednisone
Biological therapy	Rituximab may be effective for nodal marginal zone lymphoma. Interferon-alfa may be used alone or in combination with a drug called ribavirin, which is used to treat some viral infections like HCV and may be helpful.
Radiation therapy	External beam radiation therapy may be used to treat the lymph nodes in early stage (stage I or stage II) nodal marginal zone lymphoma.
Stem cell transplant	May be used in some people with nodal marginal zone lymphoma when their lymphoma recurs after treatment.