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Created page with "==Primary Author(s)*== *Snehal Patel, MD, PhD __TOC__ ==Cancer Category/Type== *Mature B-Cell Neoplasms ==Cancer Sub-Classification / Subtype== *Splenic Marginal Zone..."

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==Primary Author(s)*==

*Snehal Patel, MD, PhD
__TOC__

==Cancer Category/Type==

*[[Mature B-Cell Neoplasms]]

==Cancer Sub-Classification / Subtype==

*Splenic Marginal Zone Lymphoma (SMZL)

==Definition / Description of Disease==

*Indolent mature B-cell neoplasm of adults involving the spleen, blood, and bone marrow
*Splenic white pulp effacement by small lymphocytes and pale marginal zone involved by larger cells
*Likely originating from mature B-cells of the marginal zone
*The synonyms derive from circulating lymphocytes with cytoplasmic villous projections

==Synonyms / Terminology==

*Splenic B-cell marginal zone lymphoma
*Splenic lymphoma with villous lymphocytes
*Splenic lymphoma with circulating villous lymphocytes

==Epidemiology / Prevalence<ref name=":2" /><ref name=":6">{{Cite journal|last=Tsd|first=Santos|last2=Rs|first2=Tavares|last3=Dlc|first3=Farias|date=2017|title=Splenic Marginal Zone Lymphoma: A Literature Review of Diagnostic and Therapeutic Challenges|url=https://pubmed.ncbi.nlm.nih.gov/28577652/|language=en|doi=10.1016/j.bjhh.2016.09.014|pmc=PMC5457460|pmid=28577652}}</ref>==

*1 to 2% of lymphoid neoplasms
*Median age: mid to late 60's
*Males ~ Females

==Clinical Features<ref name=":2" /><ref name=":6" />==
'''Signs & Symptoms'''

*Splenic enlargement and discomfort
*Lymphadenopathy (rare)
*Autoimmune hemolytic anemia or thrombocytopenia
*Association with Hepatitis C virus

'''Laboratory findings'''

*Cytopenias
*Lymphocytosis (low level)
*Small paraprotein

==Sites of Involvement<ref name=":2" /><ref name=":6" />==

*Spleen (white pulp)
*Perihilar lymph nodes
*Blood
*Bone marrow
*liver (less common)
*peripheral lymph nodes (rare)

==Morphologic Features<ref name=":2" /><ref name=":6" />==

*Marked expansion splenic white pulp by neoplastic B-cells
*Effacement of mantle zone and germinal center by small neoplastic B-cells
*Residual germinal centers may be present
*Small cells and large cells with more cytoplasm are seen in the marginal zone
*Neoplastic B-cells show cytoplasmic projections in smear preparations

==Immunophenotype<ref name=":2" /><ref name=":6" />==

{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (B-cell lineage markers)||CD19, CD20 (bright), CD22, PAX5, FMC7, sIg (bright, monotypic)
|-
|Positive (subset)||CD5, CD11c, CD70, CD103
|-
|Negative||CD10, CD21, CD35, CD42, CD123, BCL1, BCL6, SOX11, LEF1, IRTA1, BRAF V600E
|}

==Chromosomal Rearrangements (Gene Fusions)==

*Rare but (some) recurrent translocations/gene fusions:
**t(8;14)(q24;q32)/IGH-MYC<ref>{{Cite journal|last=Shi|first=Xiaofeng|last2=Ba|first2=Rong|last3=You|first3=Haiyan|last4=Jiang|first4=Qian|last5=Huang|first5=Jiansong|last6=Mao|first6=Jianhua|last7=Han|first7=Lanxiu|last8=Zhang|first8=Shuo|last9=Zhuang|first9=Qin|date=2018|title=A rare case of B-lymphoproliferative disorder with villous lymphocytes harboring t(8;14)(q24;q32) translocation|url=http://link.springer.com/10.1007/s11684-017-0558-z|journal=Frontiers of Medicine|language=en|volume=12|issue=3|pages=324–329|doi=10.1007/s11684-017-0558-z|issn=2095-0217}}</ref><ref>{{Cite journal|last=Scapinello|first=Greta|last2=Pizzi|first2=Marco|last3=Vio|first3=Stefania|last4=Nabergoj|first4=Mitja|last5=Visentin|first5=Andrea|last6=Martines|first6=Annalisa|last7=Bonaldi|first7=Laura|last8=Trentin|first8=Livio|last9=Semenzato|first9=Gianpietro|date=2018|title=Splenic marginal zone lymphoma with a de novo t(8;14)(q24;q32) and a prolymphocytoid evolution responsive to rituximab-bendamustine|url=http://link.springer.com/10.1007/s00277-018-3351-4|journal=Annals of Hematology|language=en|volume=97|issue=10|pages=2001–2003|doi=10.1007/s00277-018-3351-4|issn=0939-5555}}</ref>
**KMT2A fusion in large cell transformation of SMZL<ref>{{Cite journal|last=Gindin|first=Tatyana|last2=Murty|first2=Vundavalli|last3=Alobeid|first3=Bachir|last4=Bhagat|first4=Govind|date=2015|title=MLL / KMT2A translocations in diffuse large B-cell lymphomas: MLL / KMT2A translocations in diffuse large B-cell lymphomas|url=http://doi.wiley.com/10.1002/hon.2158|journal=Hematological Oncology|language=en|volume=33|issue=4|pages=239–246|doi=10.1002/hon.2158}}</ref>
**t(2;7)(p11.2;q21.2/IGK-CDK6<ref>{{Cite journal|last=Remstein|first=E D|last2=Law|first2=M|last3=Mollejo|first3=M|last4=Piris|first4=M A|last5=Kurtin|first5=P J|last6=Dogan|first6=A|date=2008|title=The prevalence of IG translocations and 7q32 deletions in splenic marginal zone lymphoma|url=http://www.nature.com/articles/2405027|journal=Leukemia|language=en|volume=22|issue=6|pages=1268–1272|doi=10.1038/sj.leu.2405027|issn=0887-6924}}</ref><ref>{{Cite journal|last=Corcoran|first=M M|last2=Mould|first2=S J|last3=Orchard|first3=J A|last4=Ibbotson|first4=R E|last5=Chapman|first5=R M|last6=Boright|first6=A P|last7=Platt|first7=C|last8=Tsui|first8=L-C|last9=Scherer|first9=S W|date=1999|title=Dysregulation of cyclin dependent kinase 6 expression in splenic marginal zone lymphoma through chromosome 7q translocations|url=http://www.nature.com/articles/1203033|journal=Oncogene|language=en|volume=18|issue=46|pages=6271–6277|doi=10.1038/sj.onc.1203033|issn=0950-9232}}</ref>
***Also reported in CD5-negative monoclonal B-cell lymphocytosis<ref>{{Cite journal|last=Parker|first=Edward|last2=MacDonald|first2=Jeffrey R.|last3=Wang|first3=Chen|date=2011|title=Molecular characterization of a t(2;7) translocation linking CDK6 to the IGK locus in CD5− monoclonal B-cell lymphocytosis|url=https://linkinghub.elsevier.com/retrieve/pii/S2210776211000780|journal=Cancer Genetics|language=en|volume=204|issue=5|pages=260–264|doi=10.1016/j.cancergen.2011.03.004}}</ref>
**t(14;18)(q32;q21)/IGH-BCL2<ref name=":1">{{Cite journal|last=Baseggio|first=Lucile|last2=Geay|first2=Marie-Odile|last3=Gazzo|first3=Sophie|last4=Berger|first4=Françoise|last5=Traverse-Glehen|first5=Alexandra|last6=Ffrench|first6=Martine|last7=Hayette|first7=Sandrine|last8=Callet-Bauchu|first8=Evelyne|last9=Verney|first9=Aurélie|date=2012|title=In non-follicular lymphoproliferative disorders, IGH/BCL2-fusion is not restricted to chronic lymphocytic leukaemia|url=http://doi.wiley.com/10.1111/j.1365-2141.2012.09178.x|journal=British Journal of Haematology|language=en|volume=158|issue=4|pages=489–498|doi=10.1111/j.1365-2141.2012.09178.x}}</ref>
**t(5;7)(p15.33;p11)/TERT fusion<ref>{{Cite journal|last=Nagel|first=Inga|last2=Szczepanowski|first2=Monika|last3=Martín-Subero|first3=José I.|last4=Harder|first4=Lana|last5=Akasaka|first5=Takashi|last6=Ammerpohl|first6=Ole|last7=Callet-Bauchu|first7=Evelyne|last8=Gascoyne|first8=Randy D.|last9=Gesk|first9=Stefan|date=2010|title=Deregulation of the telomerase reverse transcriptase (TERT) gene by chromosomal translocations in B-cell malignancies|url=https://ashpublications.org/blood/article/116/8/1317/27904/Deregulation-of-the-telomerase-reverse|journal=Blood|language=en|volume=116|issue=8|pages=1317–1320|doi=10.1182/blood-2009-09-240440|issn=0006-4971}}</ref>
**t(9;14)(p13;q32)/IGH-PAX5<ref>{{Cite journal|last=Kelly|first=Richard J.|last2=O'Connor|first2=Sheila J. M.|last3=Barrans|first3=Sharon L.|last4=Johnson|first4=Roderick J.|last5=Owen|first5=Roger G.|date=2007|title=The t(9;14)(p13;q32) is a recurrent but rare abnormality in splenic marginal zone lymphoma|url=http://www.tandfonline.com/doi/full/10.1080/10428190701474415|journal=Leukemia & Lymphoma|language=en|volume=48|issue=8|pages=1636–1637|doi=10.1080/10428190701474415|issn=1042-8194}}</ref><ref>{{Cite journal|last=Sole|first=F.|last2=Serrano|first2=S.|last3=Colomo|first3=L.|last4=Granada|first4=I.|last5=Domingo|first5=A.|last6=Salido|first6=M.|last7=Baro|first7=C.|date=2006|title=Translocation t(9;14)(p13;q32) in cases of splenic marginal zone lymphoma|url=http://www.haematologica.org/content/91/9/1289|journal=Haematologica|language=en|volume=91|issue=9|pages=1289–1291|issn=0390-6078|pmid=16956840}}</ref><ref>{{Cite journal|last=K|first=Kawakami|last2=R|first2=Amakawa|last3=S|first3=Miyanishi|last4=A|first4=Okumura|last5=T|first5=Hayashi|last6=M|first6=Kurata|last7=H|first7=Ohno|last8=Y|first8=Ohno|last9=S|first9=Fukuhara|date=1998|title=A Case of Primary Splenic Large Cell Lymphoma With a t(9;14)(p13;q32)|url=https://pubmed.ncbi.nlm.nih.gov/9631587/|language=en|pmid=9631587}}</ref>
*

==Characteristic Chromosomal Aberrations / Patterns==

*Ig gene rearrangements

==Genomic Gain/Loss/LOH==

{| class="wikitable sortable"
|-
!Chromosome Number!!Gain/Loss/Amp/LOH
!Significance!!Prevalence
|-
|7q31-32||Loss (heterozygous)
|Unknown; possible haploinsufficiency of IRF5 tumor suppressor<ref>{{Cite journal|last=Fresquet|first=Vicente|last2=Robles|first2=Eloy F.|last3=Parker|first3=Anton|last4=Martinez-Useros|first4=Javier|last5=Mena|first5=Maria|last6=Malumbres|first6=Raquel|last7=Agirre|first7=Xabier|last8=Catarino|first8=Susana|last9=Arteta|first9=David|date=2012|title=High-throughput sequencing analysis of the chromosome 7q32 deletion reveals IRF5 as a potential tumour suppressor in splenic marginal-zone lymphoma|url=http://doi.wiley.com/10.1111/j.1365-2141.2012.09226.x|journal=British Journal of Haematology|language=en|volume=158|issue=6|pages=712–726|doi=10.1111/j.1365-2141.2012.09226.x}}</ref>||26–45%<ref name=":2">{{Cite journal|last=Salido|first=Marta|last2=Baró|first2=Cristina|last3=Oscier|first3=David|last4=Stamatopoulos|first4=Kostas|last5=Dierlamm|first5=Judith|last6=Matutes|first6=Estela|last7=Traverse-Glehen|first7=Alexandra|last8=Berger|first8=Francoise|last9=Felman|first9=Pascale|date=2010|title=Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group|url=https://ashpublications.org/blood/article/116/9/1479/103841/Cytogenetic-aberrations-and-their-prognostic-value|journal=Blood|language=en|volume=116|issue=9|pages=1479–1488|doi=10.1182/blood-2010-02-267476|issn=0006-4971}}</ref><ref name=":3">{{Cite journal|last=Baró|first=Cristina|last2=Salido|first2=Marta|last3=Espinet|first3=Blanca|last4=Astier|first4=Laura|last5=Domingo|first5=Alicia|last6=Granada|first6=Isabel|last7=Millà|first7=Fuensanta|last8=Carrió|first8=Ana|last9=Costa|first9=Dolors|date=2008|title=New chromosomal alterations in a series of 23 splenic marginal zone lymphoma patients revealed by Spectral Karyotyping (SKY)|url=https://linkinghub.elsevier.com/retrieve/pii/S0145212607003505|journal=Leukemia Research|language=en|volume=32|issue=5|pages=727–736|doi=10.1016/j.leukres.2007.09.012}}</ref><ref name=":4">{{Cite journal|last=Gruszka-Westwood|first=Alicja M.|last2=Hamoudi|first2=Rifat|last3=Osborne|first3=Lucy|last4=Matutes|first4=Estella|last5=Catovsky|first5=Daniel|date=2003|title=Deletion mapping on the long arm of chromosome 7 in splenic lymphoma with villous lymphocytes|url=http://doi.wiley.com/10.1002/gcc.10142|journal=Genes, Chromosomes and Cancer|language=en|volume=36|issue=1|pages=57–69|doi=10.1002/gcc.10142|issn=1045-2257}}</ref>
|-
|3/3q||Gain (trisomy)
|Unknown||15%<ref name=":2" /><ref name=":4" /><ref name=":3" />
|}

==Gene Mutations (SNV/INDEL)==

{| class="wikitable sortable"
|-
!Gene*!!Oncogene/Tumor Suppressor/Other!!Presumed Mechanism (LOF/GOF/Other)!!Prevalence (COSMIC)
|-
|NOTCH2||Oncogene or Tumor Suppressor<ref name=":5">{{Cite journal|last=Lobry|first=Camille|last2=Oh|first2=Philmo|last3=Aifantis|first3=Iannis|date=2011|title=Oncogenic and tumor suppressor functions of Notch in cancer: it’s NOTCH what you think|url=https://rupress.org/jem/article/208/10/1931/40940/Oncogenic-and-tumor-suppressor-functions-of-Notch|journal=The Journal of Experimental Medicine|language=en|volume=208|issue=10|pages=1931–1935|doi=10.1084/jem.20111855|issn=1540-9538|pmc=PMC3182047|pmid=21948802}}</ref>||Other
|21%
|-
|MYD88
|Oncogene
|GOF
|7%
|-
|KLF2
|Likely tumor suppressor in most contexts<ref>{{Cite journal|last=Wang|first=Chunmei|last2=Li|first2=Liang|last3=Duan|first3=Qiuhui|last4=Wang|first4=Qingqing|last5=Chen|first5=Jinlian|date=2017|title=Krüppel-like factor 2 suppresses human gastric tumorigenesis through inhibiting PTEN/AKT signaling|url=https://www.oncotarget.com/lookup/doi/10.18632/oncotarget.22229|journal=Oncotarget|language=en|volume=8|issue=59|pages=100358–100370|doi=10.18632/oncotarget.22229|issn=1949-2553|pmc=PMC5725026|pmid=29245984}}</ref>
|LOF
|20%<ref>{{Cite journal|last=Jaramillo Oquendo|first=Carolina|last2=Parker|first2=Helen|last3=Oscier|first3=David|last4=Ennis|first4=Sarah|last5=Gibson|first5=Jane|last6=Strefford|first6=Jonathan C.|date=2019|title=Systematic Review of Somatic Mutations in Splenic Marginal Zone Lymphoma|url=http://www.nature.com/articles/s41598-019-46906-1|journal=Scientific Reports|language=en|volume=9|issue=1|doi=10.1038/s41598-019-46906-1|issn=2045-2322|pmc=PMC6639539|pmid=31320741}}</ref>
|-
|TNFAIP3
|Tumor Suppressor
|LOF
|8%
|-
|TP53
|Tumor Suppressor
|LOF
|7%
|-
|BIRC3
|Oncogene or Tumor Suppressor<ref>{{Cite journal|last=Yamato|first=Azusa|last2=Soda|first2=Manabu|last3=Ueno|first3=Toshihide|last4=Kojima|first4=Shinya|last5=Sonehara|first5=Kyuto|last6=Kawazu|first6=Masahito|last7=Sai|first7=Eirin|last8=Yamashita|first8=Yoshihiro|last9=Nagase|first9=Takahide|date=2015|title=Oncogenic activity of BIRC2 and BIRC3 mutants independent of nuclear factor-κB-activating potential|url=http://doi.wiley.com/10.1111/cas.12726|journal=Cancer Science|language=en|volume=106|issue=9|pages=1137–1142|doi=10.1111/cas.12726|pmc=PMC4582982|pmid=26094954}}</ref>
|Other
|5%
|-
|CARD11
|Oncogene
|GOF
|4%
|-
|IKBKB
|Oncogene
|GOF
|4%
|-
|SPEN
|Tumor Suppressor
|LOF
|6%
|-
|NOTCH1
|Oncogene or Tumor Suppressor<ref name=":5" />
|Other
|11%
|-
|TBL1XR1
|Oncogene or Tumor Suppressor<ref>{{Cite journal|last=Cao|first=Qinghua|last2=Wang|first2=Zhuo|last3=Wang|first3=Ye|last4=Liu|first4=Fang|last5=Dong|first5=Yu|last6=Zhang|first6=Wenhui|last7=Wang|first7=Liantang|last8=Ke|first8=Zunfu|date=2018|title=TBL1XR1 promotes migration and invasion in osteosarcoma cells and is negatively regulated by miR-186-5p|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6325474/|journal=American Journal of Cancer Research|volume=8|issue=12|pages=2481–2493|issn=2156-6976|pmc=6325474|pmid=30662805}}</ref>
|Other
|7%
|-
|NFKBIE
|Tumor Suppressor
|LOF
|2%
|}
<nowiki>*</nowiki>Specific mutations in these genes may be found in [https://www.cbioportal.org/ cBioPortal] or [https://cancer.sanger.ac.uk/cosmic COSMIC].

==Epigenomics (Methylation)==

*Epigenetic dysregulation expected on basis of genetic alterations in histone modifying and chromatin remodeling factors:
**TBL1XR1 is a member of nuclear receptor corepressor (N-CoR) and histone deacetylase 3 (HDAC3) complexes
**CREBBP is a histone acetyltransferase
**ARID1A is a member of SWI-SNF complexes
**EP300 is a histone acetyltransferase
**DNMT3A is a DNA methyltransferase
*Promoter methylation and gene expression study revealed two clusters of SMZL<ref>{{Cite journal|last=Arribas|first=Alberto J.|last2=Rinaldi|first2=Andrea|last3=Mensah|first3=Afua A.|last4=Kwee|first4=Ivo|last5=Cascione|first5=Luciano|last6=Robles|first6=Eloy F.|last7=Martinez-Climent|first7=Jose A.|last8=Oscier|first8=David|last9=Arcaini|first9=Luca|date=2015|title=DNA methylation profiling identifies two splenic marginal zone lymphoma subgroups with different clinical and genetic features|url=https://ashpublications.org/blood/article/125/12/1922/33705/DNA-methylation-profiling-identifies-two-splenic|journal=Blood|language=en|volume=125|issue=12|pages=1922–1931|doi=10.1182/blood-2014-08-596247|issn=0006-4971|pmc=PMC4416938|pmid=25612624}}</ref>
**high methylation group compared to low methylation group showed
***Methylated/repressed tumor suppressor genes and unmethylated/overexpressed prosurvival genes
***Association with NOTCH2 mutations, 7q31-32 loss, and histologic transformation
***Reduced overall survival
***Reduced proliferation and reversion of phenotype in response to demethylating agents in vitro
*

==Genes and Main Pathways Involved==
{| class="wikitable"
!Molecular Feature
!Pathway
!Pathophysiologic outcome
|-
|NOTCH2, [[NOTCH1]], DTX, and SPEN mutations
|NOTCH signaling<ref name=":0">{{Cite journal|last=Rossi|first=Davide|last2=Trifonov|first2=Vladimir|last3=Fangazio|first3=Marco|last4=Bruscaggin|first4=Alessio|last5=Rasi|first5=Silvia|last6=Spina|first6=Valeria|last7=Monti|first7=Sara|last8=Vaisitti|first8=Tiziana|last9=Arruga|first9=Francesca|date=2012|title=The coding genome of splenic marginal zone lymphoma: activation of NOTCH2 and other pathways regulating marginal zone development|url=https://rupress.org/jem/article/209/9/1537/41269/The-coding-genome-of-splenic-marginal-zone|journal=The Journal of Experimental Medicine|language=en|volume=209|issue=9|pages=1537–1551|doi=10.1084/jem.20120904|issn=1540-9538|pmc=PMC3428941|pmid=22891273}}</ref>
|Increased proliferation and survival
|-
|[[MYD88]], TNFAIP3, BIRC3, CARD11, IKBKB, NFKBIE, and TRAF3 mutations
|NF-κB signaling<ref>{{Cite journal|last=Spina|first=Valeria|last2=Rossi|first2=Davide|date=2016|title=NF-κB deregulation in splenic marginal zone lymphoma|url=https://linkinghub.elsevier.com/retrieve/pii/S1044579X16300335|journal=Seminars in Cancer Biology|language=en|volume=39|pages=61–67|doi=10.1016/j.semcancer.2016.08.002}}</ref><ref>{{Cite journal|last=Yan|first=Q.|last2=Huang|first2=Y.|last3=Watkins|first3=A. J.|last4=Kocialkowski|first4=S.|last5=Zeng|first5=N.|last6=Hamoudi|first6=R. A.|last7=Isaacson|first7=P. G.|last8=de Leval|first8=L.|last9=Wotherspoon|first9=A.|date=2012|title=BCR and TLR signaling pathways are recurrently targeted by genetic changes in splenic marginal zone lymphomas|url=http://www.haematologica.org/cgi/doi/10.3324/haematol.2011.054080|journal=Haematologica|language=en|volume=97|issue=4|pages=595–598|doi=10.3324/haematol.2011.054080|issn=0390-6078|pmc=PMC3347666|pmid=22102703}}</ref><ref>{{Cite journal|last=Rossi|first=Davide|last2=Deaglio|first2=Silvia|last3=Dominguez-Sola|first3=David|last4=Rasi|first4=Silvia|last5=Vaisitti|first5=Tiziana|last6=Agostinelli|first6=Claudio|last7=Spina|first7=Valeria|last8=Bruscaggin|first8=Alessio|last9=Monti|first9=Sara|date=2011|title=Alteration of BIRC3 and multiple other NF-κB pathway genes in splenic marginal zone lymphoma|url=https://ashpublications.org/blood/article/118/18/4930/29359/Alteration-of-BIRC3-and-multiple-other-NF%CE%BAB|journal=Blood|language=en|volume=118|issue=18|pages=4930–4934|doi=10.1182/blood-2011-06-359166|issn=0006-4971}}</ref>
|Lymphocyte development
|-
|[[TP53]] mutations
|TP53 pathway
|Dysregulation of genomic stability and apoptosis
|-
|TBL1XR1, CREBBP, ARID1A, [[EP300]], and [[DNMT3A]] mutations
|Histone modification and chromatin remodeling<ref name=":0" />
|Abnormal gene expression program
|}

==Diagnostic Testing Methods==

*Clinical, morphologic, and immunophenotypic findings and exclusion of other low-grade B-cell lymphomas are generally sufficient for diagnosis
*No established specific diagnostic test currently exists

*Molecular testing may help exclude other entities in some cases (see below)

==Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications)==
{| class="wikitable"
!Alteration
!Clinical Significance
!Note
|-
|BRAF mutations
|Diagnostic (exclusion)
|Present in [[Hairy Cell Leukemia|hairy cell leukemia (HCL)]] and absent in SMZL<ref>{{Cite journal|last=Naseem|first=Shano|last2=Gupta|first2=Ojas|last3=Binota|first3=Jogeshwar|last4=Varma|first4=Neelam|last5=Varma|first5=Subhash|last6=Malhotra|first6=Pankaj|date=2020|title=BRAF V600E mutation detection in hairy cell leukemia-utility of archival DNA from bone marrow aspirate/imprint smear and amplification refractory mutation system|url=http://link.springer.com/10.1007/s11033-020-05509-0|journal=Molecular Biology Reports|language=en|doi=10.1007/s11033-020-05509-0|issn=0301-4851}}</ref>
|-
|MYD88 mutations
|Diagnostic (exclusion)
|Present in [[Lymphoplasmacytic Lymphoma|lymphoplasmacytic lymphoma (LPL)]] and rare but not absent in SMZL
|-
|t(11;14)(q13;q32)/IGH-CCND1*
|Diagnostic (exclusion)
|Present in [[Mantle Cell Lymphoma|mantle cell lymphoma (MCL)]] and absent in SMZL
|-
|t(14;18)(q32;q21)/IGH-BCL2
|Diagnostic (exclusion)
|Present in [[Follicular Lymphoma|follicular lymphoma (FL)]] and rare but not absent in SMZL<ref name=":1" />
|-
|t(11;18)(q21;q21)/BIRC3-MALT1
|Diagnostic (exclusion)
|Present in [[Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)|MALT lymphoma]] and absent in SMZL
|-
|t(14;18)(q32;q21)/IGH-MALT1
|Diagnostic (exclusion)
|Present in [[Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)|MALT lymphoma]] and absent in SMZL<ref>{{Cite journal|last=Streubel|first=Berthold|last2=Lamprecht|first2=Andrea|last3=Dierlamm|first3=Judith|last4=Cerroni|first4=Lorenzo|last5=Stolte|first5=Manfred|last6=Ott|first6=German|last7=Raderer|first7=Markus|last8=Chott|first8=Andreas|date=2003|title=T(14;18)(q32;q21) involving IGH andMALT1 is a frequent chromosomal aberration in MALT lymphoma|url=https://ashpublications.org/blood/article/101/6/2335/106539/T1418q32q21-involving-IGH-andMALT1-is-a-frequent|journal=Blood|language=en|volume=101|issue=6|pages=2335–2339|doi=10.1182/blood-2002-09-2963|issn=1528-0020}}</ref>
|-
|t(1;14)(p22;q32)/IGH-BCL10
|Diagnostic (exclusion)
|Present in [[Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)|MALT lymphoma]] and absent in SMZL
|}
<nowiki>*</nowiki>Cases previously reported as SMZL with IGH-CCND1 fusion should now be classified as MCL

==Familial Forms==

*None

==Other Information==

*None

==Links==

*[[Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
*[[Splenic Diffuse Red Pulp Small B-cell Lymphoma]]

==References==
<references />

===EXAMPLE Book===

#Arber DA, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p223-225.

==Notes==
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.

HAEM4Backup:Splenic Marginal Zone Lymphoma - Revision history

Bailey.Glen: Created page with "==Primary Author(s)*== *Snehal Patel, MD, PhD __TOC__ ==Cancer Category/Type== *Mature B-Cell Neoplasms ==Cancer Sub-Classification / Subtype== *Splenic Marginal Zone..."

Bailey.Glen: Created page with "==Primary Author(s)== Snehal Patel, MD, PhD TOC ==Cancer Category/Type== Mature B-Cell Neoplasms ==Cancer Sub-Classification / Subtype== Splenic Marginal Zone..."