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

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.[1]

Pathophysiology

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.[1]

Associated Conditions

A very small number of people with nodal marginal zone lymphoma will also have hepatitis C infection (HCV).[1]

Microscopic Pathology

On microscopic histopathological analysis, centrocytes, centroblasts, plasma cells, and immunoblasts are characteristic findings of nodal marginal zone lymphoma.

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:

Epidemiology and Demographics

Age

The incidence of nodal marginal zone lymphoma increases with age; the median age at diagnosis is 60 years.[1]

Gender

Females are more commonly affected with nodal marginal zone lymphoma than males.[1]

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.[2]

Natural History, Complications and Prognosis

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.[1]

Diagnosis

Staging

Staging for nodal marginal zone lymphoma is provided in the following table:[3]

Revised staging system for primary nodal lymphomas (Lugano classification)
Stage Involvement Extranodal (E) status
Limited
Stage I One node or a group of adjacent nodes Single extranodal lesions without nodal involvement
Stage II Two or more nodal groups on the same side of the diaphragm Stage I or II by nodal extent with limited contiguous extranodal involvement
Stage II bulky II as above with "bulky" disease Not applicable
Advanced
Stage III Nodes on both sides of the diaphragm; nodes above the diaphragm with spleen involvement Not applicable
Stage IV Additional noncontiguous extralymphatic involvement Not applicable

Symptoms

Symptoms of the nodal marginal zone lymphoma include:

Physical Examination

Vitals

HEENT

Thorax

Abdomen

Extremities

Laboratory Findings

Laboratory tests for nodal marginal zone lymphoma include:

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[1]
Therapy Description
Watchful waiting
  • May not need treatment right away unless symptomatic.
Chemotherapy
  • Nodal marginal zone lymphoma is usually sensitive to chemotherapy.
  • Monotherapy: Chlorambucil OR Fludarabine
  • Combination therapy :
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
Stem cell transplant
  • May be used in some people with nodal marginal zone lymphoma when their lymphoma recurs after treatment.

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Nodal marginal zone lymphoma . Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/nodal-marginal-zone-lymphoma/?region=nb Accessed on March 4, 2016
  2. Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Nodal+marginal+zone+lymphoma. Accessed on March 4, 2016
  3. Cheson, Bruce D.; Fisher, Richard I.; Barrington, Sally F.; Cavalli, Franco; Schwartz, Lawrence H.; Zucca, Emanuele; Lister, T. Andrew; Alliance, Australasian Leukaemia and Lymphoma Group; Eastern Cooperative Oncology Group; European Mantle Cell Lymphoma Consortium; Italian Lymphoma Foundation; European Organisation for Research; Treatment of Cancer/Dutch Hemato-Oncology Group; Grupo Español de Médula Ósea; German High-Grade Lymphoma Study Group; German Hodgkin's Study Group; Japanese Lymphorra Study Group; Lymphoma Study Association; NCIC Clinical Trials Group; Nordic Lymphoma Study Group; Southwest Oncology Group; United Kingdom National Cancer Research Institute (2014-09-20). "Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification". Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology. 32 (27): 3059–3068. ISSN 1527-7755. PMID 25113753. doi:10.1200/JCO.2013.54.8800. 

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