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'''For patient information, click [[Insert page name here (patient information)|Insert page name here]]'''
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{{CMG}}; {{AE}} {{AS}}
{{CMG}}; {{AE}} {{AS}}  {{N.F}}


{{SK}}: NMZL; Nodal marginal zone B cell lymphoma;  Monocytoid B-cell lymphoma; Nodal monocytoid B-cell lymphoma
{{SK}}: NMZL; Nodal marginal zone B cell lymphoma;  Monocytoid B-cell lymphoma; Nodal monocytoid B-cell lymphoma


== Overview ==
== Overview ==
 
Nodal marginal zone lymphoma is a rare [[B-cell]] [[neoplasm]]. Nodal marginal zone lymphoma usually only involves the [[Lymph node|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 lymphoma]]s. 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]], [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|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.
==Historical Perspective==
 
==Classification==


== Pathophysiology==
== Pathophysiology==
 
* Nodal marginal zone lymphoma is a rare [[Neoplasm|B-cell neoplasm]].
=== Genetics ===
* 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]].<ref name="canadiancancer">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 </ref>
* [[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]].<ref name="SpinaKhiabanian2016">{{cite journal|last1=Spina|first1=V.|last2=Khiabanian|first2=H.|last3=Messina|first3=M.|last4=Monti|first4=S.|last5=Cascione|first5=L.|last6=Bruscaggin|first6=A.|last7=Spaccarotella|first7=E.|last8=Holmes|first8=A. B.|last9=Arcaini|first9=L.|last10=Lucioni|first10=M.|last11=Tabbo|first11=F.|last12=Zairis|first12=S.|last13=Diop|first13=F.|last14=Cerri|first14=M.|last15=Chiaretti|first15=S.|last16=Marasca|first16=R.|last17=Ponzoni|first17=M.|last18=Deaglio|first18=S.|last19=Ramponi|first19=A.|last20=Tiacci|first20=E.|last21=Pasqualucci|first21=L.|last22=Paulli|first22=M.|last23=Falini|first23=B.|last24=Inghirami|first24=G.|last25=Bertoni|first25=F.|last26=Foa|first26=R.|last27=Rabadan|first27=R.|last28=Gaidano|first28=G.|last29=Rossi|first29=D.|title=The genetics of nodal marginal zone lymphoma|journal=Blood|volume=128|issue=10|year=2016|pages=1362–1373|issn=0006-4971|doi=10.1182/blood-2016-02-696757}}</ref>


=== Associated Conditions===
=== Associated Conditions===
 
A very small number of people with nodal marginal zone lymphoma will also have
===Gross Pathology===
* [[Hepatitis C]] infection (HCV)<ref name="canadiancancer">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 </ref>
* [[Autoimmune disorders]]


===Microscopic Pathology===
===Microscopic Pathology===
On microscopic histopathological analysis of marginal zone lymphoma.,the following features are seen:<ref name="canadiancancer" />
* Centrocytes
* Centroblasts
* [[Plasma cell|Plasma cells]]
* [[Immunoblast|Immunoblasts]]


==Causes==
==Causes==
===Life Threatening Causes===
There are no established causes for nodal marginal zone lymphoma.
Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.
==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]]
* [[Waldenström's macroglobulinemia|Waldenstrom’s macroglobulinemia]]
 
== Epidemiology and Demographics ==
===Age===
The [[incidence]] of nodal marginal zone lymphoma increases with age; the median age at diagnosis is 60 years.<ref name="canadiancancer">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 </ref>
===Gender===
Females are more commonly affected with nodal marginal zone lymphoma than males.<ref name="canadiancancer">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 </ref>
== 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.<ref name="uspreventive">Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Nodal+marginal+zone+lymphoma.  Accessed on March 4, 2016</ref>
== Natural History, Complications and Prognosis==
* Nodal marginal zone lymphoma is often slow growing (indolent)<ref name="BoveriArcaini2008">{{cite journal|last1=Boveri|first1=E.|last2=Arcaini|first2=L.|last3=Merli|first3=M.|last4=Passamonti|first4=F.|last5=Rizzi|first5=S.|last6=Vanelli|first6=L.|last7=Rumi|first7=E.|last8=Rattotti|first8=S.|last9=Lucioni|first9=M.|last10=Picone|first10=C.|last11=Castello|first11=A.|last12=Pascutto|first12=C.|last13=Magrini|first13=U.|last14=Lazzarino|first14=M.|last15=Paulli|first15=M.|title=Bone marrow histology in marginal zone B-cell lymphomas: correlation with clinical parameters and flow cytometry in 120 patients|journal=Annals of Oncology|volume=20|issue=1|year=2008|pages=129–136|issn=0923-7534|doi=10.1093/annonc/mdn563}}</ref>


===Common Causes===
* NMZL can transform into a more aggressive [[Lymphoma, large-cell|large cell lymphoma.]]
* Nodal marginal zone lymphomas behave slightly more aggressively than [[MALT lymphoma]]s.<ref name="canadiancancer">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 </ref>
* Low grade marginal zone lymphoma has a sluggish course and excellent response to early [[therapy]].
* Prognosis depends on the primary [[Organ (anatomy)|organ]] of origin.
* Those [[lymphomas]] which are positive for (11;18) [[translocation]] have the worst response to [[therapy]].


===Causes by Organ System===
== Diagnosis ==  
===Staging===
Staging for nodal marginal zone lymphoma is provided in the following table:<ref>{{Cite journal| doi = 10.1200/JCO.2013.54.8800| issn = 1527-7755| volume = 32| issue = 27| pages = 3059–3068| last1 = Cheson| first1 = Bruce D.| last2 = Fisher| first2 = Richard I.| last3 = Barrington| first3 = Sally F.| last4 = Cavalli| first4 = Franco| last5 = Schwartz| first5 = Lawrence H.| last6 = Zucca| first6 = Emanuele| last7 = Lister| first7 = T. Andrew| last8 = Alliance, Australasian Leukaemia and Lymphoma Group| last9 = Eastern Cooperative Oncology Group| last10 = European Mantle Cell Lymphoma Consortium| last11 = Italian Lymphoma Foundation| last12 = European Organisation for Research| last13 = Treatment of Cancer/Dutch Hemato-Oncology Group| last14 = Grupo Español de Médula Ósea| last15 = German High-Grade Lymphoma Study Group| last16 = German Hodgkin's Study Group| last17 = Japanese Lymphorra Study Group| last18 = Lymphoma Study Association| last19 = NCIC Clinical Trials Group| last20 = Nordic Lymphoma Study Group| last21 = Southwest Oncology Group| last22 = United Kingdom National Cancer Research Institute| title = Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification| journal = Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology| date = 2014-09-20| pmid = 25113753}}</ref>


{|style="width:80%; height:100px" border="1"
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|+ '''Revised staging system for primary nodal lymphomas (Lugano classification)'''
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | No underlying causes
! style="background: #4479BA; color:#FFF;" | Stage
|-
! style="background: #4479BA; color:#FFF;" | Involvement
|-bgcolor="LightSteelBlue"
! style="background: #4479BA; color:#FFF;" | Extranodal (E) status
| '''Chemical / poisoning'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Dermatologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Drug Side Effect'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Ear Nose Throat'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Endocrine'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Environmental'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Gastroenterologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Genetic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Hematologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Iatrogenic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Infectious Disease'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Musculoskeletal / Ortho'''
|bgcolor="Beige"| No underlying causes
|-
|-
|-bgcolor="LightSteelBlue"
| colspan="3" style="padding: 5px 5px; background: #DCDCDC;" | '''Limited'''
| '''Neurologic'''
|bgcolor="Beige"| No underlying causes
|-
|-
|-bgcolor="LightSteelBlue"
| style="padding: 5px 5px; background: #F5F5F5;" | Stage I
| '''Nutritional / Metabolic'''
| style="padding: 5px 5px; background: #F5F5F5;" | One node or a group of adjacent nodes
|bgcolor="Beige"| No underlying causes
| style="padding: 5px 5px; background: #F5F5F5;" | Single extranodal lesions without nodal involvement
|-
|-
|-bgcolor="LightSteelBlue"
| style="padding: 5px 5px; background: #F5F5F5;" | Stage II
| '''Obstetric/Gynecologic'''
| style="padding: 5px 5px; background: #F5F5F5;" | Two or more nodal groups on the same side of the diaphragm
|bgcolor="Beige"| No underlying causes
| style="padding: 5px 5px; background: #F5F5F5;" | Stage I or II by nodal extent with limited contiguous extranodal involvement
|-
|-
|-bgcolor="LightSteelBlue"
| style="padding: 5px 5px; background: #F5F5F5;" | Stage II bulky
| '''Oncologic'''
| style="padding: 5px 5px; background: #F5F5F5;" | II as above with "bulky" disease
|bgcolor="Beige"| No underlying causes
| style="padding: 5px 5px; background: #F5F5F5;" | Not applicable
|-
|-
|-bgcolor="LightSteelBlue"
| colspan="3" style="padding: 5px 5px; background: #DCDCDC;" | '''Advanced'''
| '''Opthalmologic'''
|bgcolor="Beige"| No underlying causes
|-
|-
|-bgcolor="LightSteelBlue"
| style="padding: 5px 5px; background: #F5F5F5;" | Stage III
| '''Overdose / Toxicity'''
| style="padding: 5px 5px; background: #F5F5F5;" | Nodes on both sides of the diaphragm; nodes above the diaphragm with spleen involvement
|bgcolor="Beige"| No underlying causes
| style="padding: 5px 5px; background: #F5F5F5;" | Not applicable
|-
|-bgcolor="LightSteelBlue"
| '''Psychiatric'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Pulmonary'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Renal / Electrolyte'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Rheum / Immune / Allergy'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Sexual'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Trauma'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Urologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Dental'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Miscellaneous'''
|bgcolor="Beige"| No underlying causes
|-
|-
| style="padding: 5px 5px; background: #F5F5F5;" | Stage IV
| style="padding: 5px 5px; background: #F5F5F5;" | Additional noncontiguous extralymphatic involvement
| style="padding: 5px 5px; background: #F5F5F5;" | Not applicable
|}
|}
===Causes in Alphabetical Order===
*A...
*Z...
Make sure that each diagnosis is linked to a page.
==Differentiating type page name here from other Diseases==
== Epidemiology and Demographics ==
===Age===
===Gender===
===Race===
===Developed Countries===
===Developing Countries===
== Risk Factors ==
== Screening ==
== Natural History, Complications and Prognosis==
== Diagnosis ==
===Diagnostic Criteria===
If available, the diagnostic criteria are provided here.
===History===
A directed history should be obtained to ascertain


=== Symptoms ===
=== Symptoms ===
"Type symptom here" is pathognomonic of the "type disease name here".
Symptoms of the nodal marginal zone lymphoma include:
 
* [[Fever]]
"Type non specific symptoms" may be present.
* [[Weight loss]]
 
* [[Night sweats]]
===Past Medical History===
* Painless [[swelling]] in the neck, [[axilla]], [[groin]], [[thorax]], and [[abdomen]]
 
===Family History===
 
===Social History===
====Occupational====
====Alcohol====
The frequency and amount of alcohol consumption should be characterized.
====Drug Use====
====Smoking====
 
===Allergies===
 
=== Physical Examination ===
=== Physical Examination ===
====Vitals====
* [[Fever]] is often present
====HEENT====
* [[Lymphadenopathy|Cervical lymphadenopathy]]
====Thorax====
* Thoracic masses suggestive of [[Lymphadenopathy|central lymphadenopathy]]
====Abdomen====
* [[Abdominal mass]]es suggestive of [[Lymphadenopathy|central lymphadenopathy]]
====Extremities====
* [[Lymphadenopathy|Peripheral lymphadenopathy]]


==== Appearance of the Patient ====
===Laboratory Findings ===  
 
Laboratory tests for nodal marginal zone lymphoma include:
====Vital Signs====
* [[Complete blood count]] (CBC)
 
* [[Blood chemistry tests|Blood chemistry]] studies
====Skin====
* [[Cytogenetic analysis]]
 
* [[Flow cytometry]]
====Head====
* [[Immunohistochemistry]]
 
* [[Immunophenotyping]]:
==== Eyes ====
:*  Positive: [[CD19]], [[CD79a]], [[bcl2]], and  [[CD22]]
 
:* Negative: [[CD5]], [[CD10]], [[CD23]], [[CD25]], [[bcl10]], and [[cyclin D1]]
==== Ear ====
====Biopsy====
 
[[Lymph node]] or [[bone marrow biopsy]] is diagnostic of nodal marginal zone lymphoma.
====Nose====
 
====Mouth====
 
====Throat ====
 
==== Heart ====
 
==== Lungs ====
 
==== Abdomen ====
 
==== Extremities ====
 
==== Neurologic ====
 
====Genitals====
 
==== Other ====
 
=== Laboratory Findings ===
 
==== Electrolyte and Biomarker Studies ====
 
==== Electrocardiogram ====
 
==== Chest X Ray ====
 
====CT ====
====CT ====
 
[[CT]] scan may be helpful in the [[diagnosis]] of nodal marginal zone lymphoma.
==== MRI ====
==== MRI ====
 
[[MRI]] scan may be helpful in the [[diagnosis]] of nodal marginal zone lymphoma.
==== Echocardiography or Ultrasound ====
 
==== Other Imaging Findings ====
==== Other Imaging Findings ====
[[PET]] scan may be helpful in the diagnosis of nodal marginal zone lymphoma.


=== Other Diagnostic Studies ===
==Treatment==
===Medical Therapy===


== Treatment ==
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
=== Pharmacotherapy ===
|+ '''Treatment of nodal marginal zone lymphoma<ref name="canadiancancer">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 </ref>'''
 
! style="background: #4479BA; color:#FFF;" | Therapy
==== Acute Pharmacotherapies ====
! style="background: #4479BA; color:#FFF;" | Description
 
|-
==== Chronic Pharmacotherapies ====
| style="padding: 5px 5px; background: #DCDCDC;" | Watchful waiting
 
| style="padding: 5px 5px; background: #F5F5F5;" | 
=== Surgery and Device Based Therapy ===
* May not need treatment right away unless symptomatic.
 
|-
==== Indications for Surgery ====
| style="padding: 5px 5px; background: #DCDCDC;" | [[Chemotherapy]]
 
| style="padding: 5px 5px; background: #F5F5F5;" |
==== Pre-Operative Assessment ====
* Nodal marginal zone lymphoma is usually sensitive to chemotherapy.
 
* Monotherapy: [[Chlorambucil]] {{or}} [[Fludarabine]]
==== Post-Operative Management ====
* Combination therapy :
 
:* CVP – [[Cyclophosphamide]] {{and}} [[Vincristine]] {{and}} [[Prednisone]]
==== Transplantation ====
:* CHOP – [[Cyclophosphamide]] {{and}} [[Doxorubicin]] {{and}} [[Vincristine]] {{and}} [[Prednisone]]
 
|-
=== Primary Prevention ===
| style="padding: 5px 5px; background: #DCDCDC;" | [[Biological therapy]]
 
| style="padding: 5px 5px; background: #F5F5F5;" |
=== Secondary Prevention ===
* [[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.
=== Cost-Effectiveness of Therapy ===
|-
 
| style="padding: 5px 5px; background: #DCDCDC;" | [[Radiation therapy]]
=== Future or Investigational Therapies ===
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[External beam radiation therapy]] may be used to treat the [[lymph nodes]] in early stage (stage I or stage II) nodal marginal zone lymphoma.
|-
| style="padding: 5px 5px; background: #DCDCDC;" | [[Stem cell transplant]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* May be used in some people with nodal marginal zone lymphoma when their [[Lymphomas|lymphoma]] recurs after treatment.
|}


==References==
==References==
Line 274: Line 169:


[[Category:Disease]]
[[Category:Disease]]
[[Category:Oncology]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Hematology]]
[[Category:Immunology]]

Latest revision as of 14:25, 9 January 2019

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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] Nazia Fuad M.D.

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

Associated Conditions

A very small number of people with nodal marginal zone lymphoma will also have

Microscopic Pathology

On microscopic histopathological analysis of marginal zone lymphoma.,the following features are seen:[1]

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.[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]

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. ↑ 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.
  3. ↑ Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Nodal+marginal+zone+lymphoma. Accessed on March 4, 2016
  4. ↑ 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.
  5. ↑ 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.
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