Nodal marginal zone lymphoma: Difference between revisions
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'''For patient information, click [[Insert page name here (patient information)|Insert page name here]]''' | '''For patient information, click [[Insert page name here (patient information)|Insert page name here]]''' | ||
{{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 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. | 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. | ||
== Pathophysiology== | == 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.<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> | * Nodal marginal zone lymphoma is a rare [[Neoplasm|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]].<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 [[ | A very small number of people with nodal marginal zone lymphoma will also have | ||
* [[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 | 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== | ||
There are no established causes for nodal marginal zone lymphoma. | There are no established causes for nodal marginal zone lymphoma. | ||
==Differentiating | ==Differentiating Nodal Marginal Zone Lymphoma from other Diseases== | ||
Nodal marginal zone lymphoma must be differentiated from other diseases such as: | Nodal marginal zone lymphoma must be differentiated from other diseases such as: | ||
* [[Chronic lymphocytic leukemia]] | * [[Chronic lymphocytic leukemia]] | ||
| Line 25: | Line 38: | ||
* [[Mantle cell lymphoma]] | * [[Mantle cell lymphoma]] | ||
* [[Diffuse large B cell lymphoma]] (DLBCL) | * [[Diffuse large B cell lymphoma]] (DLBCL) | ||
* [[Lymphoplasmacytic lymphoma]] | |||
* [[Waldenström's macroglobulinemia|Waldenstrom’s macroglobulinemia]] | |||
== Epidemiology and Demographics == | == Epidemiology and Demographics == | ||
===Age=== | ===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> | 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=== | ===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> | 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 == | == Risk Factors == | ||
There are no established risk factors for nodal marginal zone lymphoma. | There are no established risk factors for nodal marginal zone lymphoma. | ||
== Screening == | == 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> | 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== | == Natural History, Complications and Prognosis== | ||
Nodal marginal zone lymphoma is often slow growing (indolent). | * 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> | ||
* 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]]. | |||
== Diagnosis == | == Diagnosis == | ||
===Staging=== | ===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> | 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="border: 0px; font-size: 90%; margin: 3px;" align=center | {| style="border: 0px; font-size: 90%; margin: 3px;" align="center" | ||
|+ '''Revised staging system for primary nodal lymphomas (Lugano classification)''' | |+ '''Revised staging system for primary nodal lymphomas (Lugano classification)''' | ||
! style="background: #4479BA; color:#FFF;" | Stage | ! style="background: #4479BA; color:#FFF;" | Stage | ||
| Line 46: | Line 69: | ||
! style="background: #4479BA; color:#FFF;" | Extranodal (E) status | ! style="background: #4479BA; color:#FFF;" | Extranodal (E) status | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;" | | colspan="3" style="padding: 5px 5px; background: #DCDCDC;" | '''Limited''' | ||
|- | |- | ||
| style="padding: 5px 5px; background: #F5F5F5;" | Stage I | | style="padding: 5px 5px; background: #F5F5F5;" | Stage I | ||
| Line 60: | Line 83: | ||
| style="padding: 5px 5px; background: #F5F5F5;" | Not applicable | | style="padding: 5px 5px; background: #F5F5F5;" | Not applicable | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;" | | colspan="3" style="padding: 5px 5px; background: #DCDCDC;" | '''Advanced''' | ||
|- | |- | ||
| style="padding: 5px 5px; background: #F5F5F5;" | Stage III | | style="padding: 5px 5px; background: #F5F5F5;" | Stage III | ||
| Line 76: | Line 99: | ||
* [[Weight loss]] | * [[Weight loss]] | ||
* [[Night sweats]] | * [[Night sweats]] | ||
* Painless swelling in the neck, axilla, groin, thorax, and abdomen | * Painless [[swelling]] in the neck, [[axilla]], [[groin]], [[thorax]], and [[abdomen]] | ||
=== Physical Examination === | === Physical Examination === | ||
====Vitals==== | ====Vitals==== | ||
| Line 92: | Line 115: | ||
Laboratory tests for nodal marginal zone lymphoma include: | Laboratory tests for nodal marginal zone lymphoma include: | ||
* [[Complete blood count]] (CBC) | * [[Complete blood count]] (CBC) | ||
* Blood chemistry studies | * [[Blood chemistry tests|Blood chemistry]] studies | ||
* Cytogenetic analysis | * [[Cytogenetic analysis]] | ||
* [[Flow cytometry]] | * [[Flow cytometry]] | ||
* [[Immunohistochemistry]] | * [[Immunohistochemistry]] | ||
| Line 100: | Line 123: | ||
:* Negative: [[CD5]], [[CD10]], [[CD23]], [[CD25]], [[bcl10]], and [[cyclin D1]] | :* Negative: [[CD5]], [[CD10]], [[CD23]], [[CD25]], [[bcl10]], and [[cyclin D1]] | ||
====Biopsy==== | ====Biopsy==== | ||
Lymph node or bone marrow biopsy is diagnostic of nodal marginal zone lymphoma. | [[Lymph node]] or [[bone marrow biopsy]] is diagnostic of nodal marginal zone lymphoma. | ||
====CT ==== | ====CT ==== | ||
[[CT]] scan may be helpful in the diagnosis of nodal marginal zone lymphoma. | [[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. | [[MRI]] scan may be helpful in the [[diagnosis]] of nodal marginal zone lymphoma. | ||
==== Other Imaging Findings ==== | ==== Other Imaging Findings ==== | ||
[[PET]] scan may be helpful in the diagnosis of nodal marginal zone lymphoma. | [[PET]] scan may be helpful in the diagnosis of nodal marginal zone lymphoma. | ||
==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center | {| style="border: 0px; font-size: 90%; margin: 3px;" align="center" | ||
|+ '''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> | |+ '''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 | ! style="background: #4479BA; color:#FFF;" | Therapy | ||
! style="background: #4479BA; color:#FFF;" | Description | ! style="background: #4479BA; color:#FFF;" | Description | ||
| Line 117: | Line 141: | ||
| style="padding: 5px 5px; background: #DCDCDC;" | Watchful waiting | | style="padding: 5px 5px; background: #DCDCDC;" | Watchful waiting | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
* May not need treatment right away unless | * May not need treatment right away unless symptomatic. | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;" | [[Chemotherapy]] | | style="padding: 5px 5px; background: #DCDCDC;" | [[Chemotherapy]] | ||
| Line 134: | Line 158: | ||
| style="padding: 5px 5px; background: #DCDCDC;" | [[Radiation therapy]] | | style="padding: 5px 5px; background: #DCDCDC;" | [[Radiation therapy]] | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | 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. | * [[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: #DCDCDC;" | [[Stem cell transplant]] | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
* May be used in some people with nodal marginal zone lymphoma when their lymphoma recurs after treatment. | * May be used in some people with nodal marginal zone lymphoma when their [[Lymphomas|lymphoma]] recurs after treatment. | ||
|} | |} | ||
| Line 146: | Line 170: | ||
[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Oncology]] | [[Category:Oncology]] | ||
[[Category:Up-To-Date]] | |||
[[Category:Oncology]] | |||
[[Category:Medicine]] | |||
[[Category:Hematology]] | |||
[[Category:Immunology]] | |||
Latest revision as of 14:25, 9 January 2019
For patient information, click Insert page name here
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
- 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:[1]
- 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
- 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.[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.