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.
- 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.[1]
- 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.[2]
Associated Conditions
A very small number of people with nodal marginal zone lymphoma will also have
- Hepatitis C infection (HCV).[1]
- 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)
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.[3]
Natural History, Complications and Prognosis
- Nodal marginal zone lymphoma is often slow growing (indolent)[4]
- NMZL can transform into a more aggressive large cell lymphoma.
- Nodal marginal zone lymphomas behave slightly more aggressively than MALT lymphomas.[1]
- 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:[5]
| 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:
- Fever
- Weight loss
- Night sweats
- Painless swelling in the neck, axilla, groin, thorax, and abdomen
Physical Examination
Vitals
- Fever is often present
HEENT
Thorax
- Thoracic masses suggestive of central lymphadenopathy
Abdomen
- Abdominal masses suggestive of central lymphadenopathy
Extremities
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
| Therapy | Description |
|---|---|
| Watchful waiting |
|
| Chemotherapy |
|
| Biological therapy |
|
| Radiation therapy |
|
| Stem cell transplant |
|
References
- ↑ 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
- ↑ Spina, V.; Khiabanian, H.; Messina, M.; Monti, S.; Cascione, L.; Bruscaggin, A.; Spaccarotella, E.; Holmes, A. B.; Arcaini, L.; Lucioni, M.; Tabbo, F.; Zairis, S.; Diop, F.; Cerri, M.; Chiaretti, S.; Marasca, R.; Ponzoni, M.; Deaglio, S.; Ramponi, A.; Tiacci, E.; Pasqualucci, L.; Paulli, M.; Falini, B.; Inghirami, G.; Bertoni, F.; Foa, R.; Rabadan, R.; Gaidano, G.; Rossi, D. (2016). "The genetics of nodal marginal zone lymphoma". Blood. 128 (10): 1362–1373. doi:10.1182/blood-2016-02-696757. ISSN 0006-4971.
- ↑ Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Nodal+marginal+zone+lymphoma. Accessed on March 4, 2016
- ↑ Boveri, E.; Arcaini, L.; Merli, M.; Passamonti, F.; Rizzi, S.; Vanelli, L.; Rumi, E.; Rattotti, S.; Lucioni, M.; Picone, C.; Castello, A.; Pascutto, C.; Magrini, U.; Lazzarino, M.; Paulli, M. (2008). "Bone marrow histology in marginal zone B-cell lymphomas: correlation with clinical parameters and flow cytometry in 120 patients". Annals of Oncology. 20 (1): 129–136. doi:10.1093/annonc/mdn563. ISSN 0923-7534.
- ↑ 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. doi:10.1200/JCO.2013.54.8800. ISSN 1527-7755. PMID 25113753.