Splenic marginal zone lymphoma differential diagnosis: Difference between revisions

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{{Splenic marginal zone lymphoma}}
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{{CMG}}; {{AE}}{{Affan}}, {{AS}}
==Overview==
[[Splenic marginal zone lymphoma]] ([[SMZL]]) must be differentiated from other [[B-cell]] [[lymphomas]] such as [[chronic lymphocytic leukemia]],  [[follicular lymphoma]], [[mantle cell lymphoma]] and unclassifiable B-cell lymphomas including Hairy cell leukemia variant (HCL-v) and splenic diffuse red pulp small B-cell lymphoma (SDRPL) on the basis of cytogenetics, [[immunophenotyping]] and morphological as the treatment for these conditions varies.
==Differential Diagnosis==
Splenic marginal zone lymphoma(SMZL) must be differentiated from other splenic B-cell lymphomas with the help of clincal, morphological, immunophenotypic and genetic data as the treatment of all these conditions are different from each other.<ref name="pmid18094718">{{cite journal |vauthors=Matutes E, Oscier D, Montalban C, Berger F, Callet-Bauchu E, Dogan A, Felman P, Franco V, Iannitto E, Mollejo M, Papadaki T, Remstein ED, Salar A, Solé F, Stamatopoulos K, Thieblemont C, Traverse-Glehen A, Wotherspoon A, Coiffier B, Piris MA |title=Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria |journal=Leukemia |volume=22 |issue=3 |pages=487–95 |date=March 2008 |pmid=18094718 |doi=10.1038/sj.leu.2405068 |url=}}</ref><ref name="pmid20479288">{{cite journal |vauthors=Salido M, Baró C, Oscier D, Stamatopoulos K, Dierlamm J, Matutes E, Traverse-Glehen A, Berger F, Felman P, Thieblemont C, Gesk S, Athanasiadou A, Davis Z, Gardiner A, Milla F, Ferrer A, Mollejo M, Calasanz MJ, Florensa L, Espinet B, Luño E, Wlodarska I, Verhoef G, García-Granero M, Salar A, Papadaki T, Serrano S, Piris MA, Solé F |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 |journal=Blood |volume=116 |issue=9 |pages=1479–88 |date=September 2010 |pmid=20479288 |doi=10.1182/blood-2010-02-267476 |url=}}</ref><ref name="pmid22891276">{{cite journal |vauthors=Kiel MJ, Velusamy T, Betz BL, Zhao L, Weigelin HG, Chiang MY, Huebner-Chan DR, Bailey NG, Yang DT, Bhagat G, Miranda RN, Bahler DW, Medeiros LJ, Lim MS, Elenitoba-Johnson KS |title=Whole-genome sequencing identifies recurrent somatic NOTCH2 mutations in splenic marginal zone lymphoma |journal=J. Exp. Med. |volume=209 |issue=9 |pages=1553–65 |date=August 2012 |pmid=22891276 |pmc=3428949 |doi=10.1084/jem.20120910 |url=}}</ref><ref name="pmid22891273">{{cite journal |vauthors=Rossi D, Trifonov V, Fangazio M, Bruscaggin A, Rasi S, Spina V, Monti S, Vaisitti T, Arruga F, Famà R, Ciardullo C, Greco M, Cresta S, Piranda D, Holmes A, Fabbri G, Messina M, Rinaldi A, Wang J, Agostinelli C, Piccaluga PP, Lucioni M, Tabbò F, Serra R, Franceschetti S, Deambrogi C, Daniele G, Gattei V, Marasca R, Facchetti F, Arcaini L, Inghirami G, Bertoni F, Pileri SA, Deaglio S, Foà R, Dalla-Favera R, Pasqualucci L, Rabadan R, Gaidano G |title=The coding genome of splenic marginal zone lymphoma: activation of NOTCH2 and other pathways regulating marginal zone development |journal=J. Exp. Med. |volume=209 |issue=9 |pages=1537–51 |date=August 2012 |pmid=22891273 |pmc=3428941 |doi=10.1084/jem.20120904 |url=}}</ref><ref name="pmid21663470">{{cite journal |vauthors=Tiacci E, Trifonov V, Schiavoni G, Holmes A, Kern W, Martelli MP, Pucciarini A, Bigerna B, Pacini R, Wells VA, Sportoletti P, Pettirossi V, Mannucci R, Elliott O, Liso A, Ambrosetti A, Pulsoni A, Forconi F, Trentin L, Semenzato G, Inghirami G, Capponi M, Di Raimondo F, Patti C, Arcaini L, Musto P, Pileri S, Haferlach C, Schnittger S, Pizzolo G, Foà R, Farinelli L, Haferlach T, Pasqualucci L, Rabadan R, Falini B |title=BRAF mutations in hairy-cell leukemia |journal=N. Engl. J. Med. |volume=364 |issue=24 |pages=2305–15 |date=June 2011 |pmid=21663470 |doi=10.1056/NEJMoa1014209 |url=}}</ref><ref name="pmid24241536">{{cite journal |vauthors=Waterfall JJ, Arons E, Walker RL, Pineda M, Roth L, Killian JK, Abaan OD, Davis SR, Kreitman RJ, Meltzer PS |title=High prevalence of MAP2K1 mutations in variant and IGHV4-34-expressing hairy-cell leukemias |journal=Nat. Genet. |volume=46 |issue=1 |pages=8–10 |date=January 2014 |pmid=24241536 |pmc=3905739 |doi=10.1038/ng.2828 |url=}}</ref><ref name="pmid18042795">{{cite journal |vauthors=Traverse-Glehen A, Baseggio L, Bauchu EC, Morel D, Gazzo S, Ffrench M, Verney A, Rolland D, Thieblemont C, Magaud JP, Salles G, Coiffier B, Berger F, Felman P |title=Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity? |journal=Blood |volume=111 |issue=4 |pages=2253–60 |date=February 2008 |pmid=18042795 |doi=10.1182/blood-2007-07-098848 |url=}}</ref><ref name="pmid22301699">{{cite journal |vauthors=Braggio E, Dogan A, Keats JJ, Chng WJ, Huang G, Matthews JM, Maurer MJ, Law ME, Bosler DS, Barrett M, Lossos IS, Witzig TE, Fonseca R |title=Genomic analysis of marginal zone and lymphoplasmacytic lymphomas identified common and disease-specific abnormalities |journal=Mod. Pathol. |volume=25 |issue=5 |pages=651–60 |date=May 2012 |pmid=22301699 |pmc=3341516 |doi=10.1038/modpathol.2011.213 |url=}}</ref><ref name="pmid23611359">{{cite journal |vauthors=Traverse-Glehen A, Bachy E, Baseggio L, Callet-Bauchu E, Gazzo S, Verney A, Hayette S, Jallades L, Ffrench M, Salles G, Coiffier B, Felman P, Berger F |title=Immunoarchitectural patterns in splenic marginal zone lymphoma: correlations with chromosomal aberrations, IGHV mutations, and survival. A study of 76 cases |journal=Histopathology |volume=62 |issue=6 |pages=876–93 |date=May 2013 |pmid=23611359 |doi=10.1111/his.12092 |url=}}</ref><ref name="pmid22210875">{{cite journal |vauthors=Xi L, Arons E, Navarro W, Calvo KR, Stetler-Stevenson M, Raffeld M, Kreitman RJ |title=Both variant and IGHV4-34-expressing hairy cell leukemia lack the BRAF V600E mutation |journal=Blood |volume=119 |issue=14 |pages=3330–2 |date=April 2012 |pmid=22210875 |pmc=3321859 |doi=10.1182/blood-2011-09-379339 |url=}}</ref><ref name="pmid8123845">{{cite journal |vauthors=Matutes E, Morilla R, Owusu-Ankomah K, Houlihan A, Catovsky D |title=The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B-cell disorders |journal=Blood |volume=83 |issue=6 |pages=1558–62 |date=March 1994 |pmid=8123845 |doi= |url=}}</ref><ref name="pmid21917686">{{cite journal |vauthors=Venkataraman G, Aguhar C, Kreitman RJ, Yuan CM, Stetler-Stevenson M |title=Characteristic CD103 and CD123 expression pattern defines hairy cell leukemia: usefulness of CD123 and CD103 in the diagnosis of mature B-cell lymphoproliferative disorders |journal=Am. J. Clin. Pathol. |volume=136 |issue=4 |pages=625–30 |date=October 2011 |pmid=21917686 |doi=10.1309/AJCPKUM9J4IXCWEU |url=}}</ref><ref name="pmid23073320">{{cite journal |vauthors=Ponzoni M, Kanellis G, Pouliou E, Baliakas P, Scarfò L, Ferreri AJ, Doglioni C, Bikos V, Dagklis A, Anagnostopoulos A, Ghia P, Stamatopoulos K, Papadaki T |title=Bone marrow histopathology in the diagnostic evaluation of splenic marginal-zone and splenic diffuse red pulp small B-cell lymphoma: a reliable substitute for spleen histopathology? |journal=Am. J. Surg. Pathol. |volume=36 |issue=11 |pages=1609–18 |date=November 2012 |pmid=23073320 |doi=10.1097/PAS.0b013e318271243d |url=}}</ref><ref name="pmid15183626">{{cite journal |vauthors=Falini B, Tiacci E, Liso A, Basso K, Sabattini E, Pacini R, Foa R, Pulsoni A, Dalla Favera R, Pileri S |title=Simple diagnostic assay for hairy cell leukaemia by immunocytochemical detection of annexin A1 (ANXA1) |journal=Lancet |volume=363 |issue=9424 |pages=1869–70 |date=June 2004 |pmid=15183626 |doi=10.1016/S0140-6736(04)16356-3 |url=}}</ref><ref name="pmid20220064">{{cite journal |vauthors=Kanellis G, Mollejo M, Montes-Moreno S, Rodriguez-Pinilla SM, Cigudosa JC, Algara P, Montalban C, Matutes E, Wotherspoon A, Piris MA |title=Splenic diffuse red pulp small B-cell lymphoma: revision of a series of cases reveals characteristic clinico-pathological features |journal=Haematologica |volume=95 |issue=7 |pages=1122–9 |date=July 2010 |pmid=20220064 |doi=10.3324/haematol.2009.013714 |url=}}</ref><ref name="pmid24366360">{{cite journal |vauthors=Hunter ZR, Xu L, Yang G, Zhou Y, Liu X, Cao Y, Manning RJ, Tripsas C, Patterson CJ, Sheehy P, Treon SP |title=The genomic landscape of Waldenstrom macroglobulinemia is characterized by highly recurring MYD88 and WHIM-like CXCR4 mutations, and small somatic deletions associated with B-cell lymphomagenesis |journal=Blood |volume=123 |issue=11 |pages=1637–46 |date=March 2014 |pmid=24366360 |doi=10.1182/blood-2013-09-525808 |url=}}</ref><ref name="pmid15767800">{{cite journal |vauthors=Went PT, Zimpfer A, Pehrs AC, Sabattini E, Pileri SA, Maurer R, Terracciano L, Tzankov A, Sauter G, Dirnhofer S |title=High specificity of combined TRAP and DBA.44 expression for hairy cell leukemia |journal=Am. J. Surg. Pathol. |volume=29 |issue=4 |pages=474–8 |date=April 2005 |pmid=15767800 |doi= |url=}}</ref><ref name="pmid19287458">{{cite journal |vauthors=Morice WG, Chen D, Kurtin PJ, Hanson CA, McPhail ED |title=Novel immunophenotypic features of marrow lymphoplasmacytic lymphoma and correlation with Waldenström's macroglobulinemia |journal=Mod. Pathol. |volume=22 |issue=6 |pages=807–16 |date=June 2009 |pmid=19287458 |doi=10.1038/modpathol.2009.34 |url=}}</ref><ref name="pmid25268192">{{cite journal |vauthors=Behdad A, Bailey NG |title=Diagnosis of splenic B-cell lymphomas in the bone marrow: a review of histopathologic, immunophenotypic, and genetic findings |journal=Arch. Pathol. Lab. Med. |volume=138 |issue=10 |pages=1295–301 |date=October 2014 |pmid=25268192 |doi=10.5858/arpa.2014-0291-CC |url=}}</ref><ref name="pmid23347903">{{cite journal |vauthors=Shao H, Calvo KR, Grönborg M, Tembhare PR, Kreitman RJ, Stetler-Stevenson M, Yuan CM |title=Distinguishing hairy cell leukemia variant from hairy cell leukemia: development and validation of diagnostic criteria |journal=Leuk. Res. |volume=37 |issue=4 |pages=401–409 |date=April 2013 |pmid=23347903 |pmc=5575750 |doi=10.1016/j.leukres.2012.11.021 |url=}}</ref><ref name="pmid25114404">{{cite journal |vauthors=Konkay K, Uppin MS, Uppin SG, Raghunadha Rao D, Geetha Ch, Paul TR |title=Hairy cell leukemia: clinicopathological and immunophenotypic study |journal=Indian J Hematol Blood Transfus |volume=30 |issue=3 |pages=180–6 |date=September 2014 |pmid=25114404 |pmc=4115087 |doi=10.1007/s12288-013-0231-x |url=}}</ref><ref name="pmid25818826">{{cite journal |vauthors=Rudolf-Oliveira RC, Pirolli MM, de Souza FS, Michels J, Santos-Silva MC |title=Hairy cell leukemia variant: the importance of differential diagnosis |journal=Rev Bras Hematol Hemoter |volume=37 |issue=2 |pages=132–5 |date=2015 |pmid=25818826 |pmc=4382572 |doi=10.1016/j.bjhh.2015.01.003 |url=}}</ref><ref name="pmid19880776">{{cite journal |vauthors=Pileri SA, Falini B |title=Mantle cell lymphoma |journal=Haematologica |volume=94 |issue=11 |pages=1488–92 |date=November 2009 |pmid=19880776 |pmc=2770958 |doi=10.3324/haematol.2009.013359 |url=}}</ref><ref name="pmid22541613">{{cite journal |vauthors=Traverse-Glehen A, Baseggio L, Salles G, Coiffier B, Felman P, Berger F |title=Splenic diffuse red pulp small-B cell lymphoma: toward the emergence of a new lymphoma entity |journal=Discov Med |volume=13 |issue=71 |pages=253–65 |date=April 2012 |pmid=22541613 |doi= |url=}}</ref><ref name="pmid22499304">{{cite journal |vauthors=Rahman K, Subramanian PG, Kadam PA, Gadage V, Galani K, Mittal N, Ghogale S, Badrinath Y, Ansari R, Kushte S, Nair R, Sengar M, Menon H, Gujral S |title=Morphological spectrum of leukemic mantle cell lymphoma |journal=Indian J Pathol Microbiol |volume=55 |issue=1 |pages=66–71 |date=2012 |pmid=22499304 |doi=10.4103/0377-4929.94860 |url=}}</ref><ref name="pmid15762289">{{cite journal |vauthors=Cessna MH, Hartung L, Tripp S, Perkins SL, Bahler DW |title=Hairy cell leukemia variant: fact or fiction |journal=Am. J. Clin. Pathol. |volume=123 |issue=1 |pages=132–8 |date=January 2005 |pmid=15762289 |doi= |url=}}</ref><ref name="pmid25818826">{{cite journal |vauthors=Rudolf-Oliveira RC, Pirolli MM, de Souza FS, Michels J, Santos-Silva MC |title=Hairy cell leukemia variant: the importance of differential diagnosis |journal=Rev Bras Hematol Hemoter |volume=37 |issue=2 |pages=132–5 |date=2015 |pmid=25818826 |pmc=4382572 |doi=10.1016/j.bjhh.2015.01.003 |url=}}</ref><ref name="pmid11789727">{{cite journal |vauthors=Kansal R, Singleton TP, Ross CW, Finn WG, Padmore RF, Schnitzer B |title=Follicular hodgkin lymphoma: a histopathologic study |journal=Am. J. Clin. Pathol. |volume=117 |issue=1 |pages=29–35 |date=January 2002 |pmid=11789727 |doi=10.1309/M7YV-V8V2-A5VA-J1Y4 |url=}}</ref><ref name="pmid29748447">{{cite journal |vauthors=Autore F, Strati P, Laurenti L, Ferrajoli A |title=Morphological, immunophenotypic, and genetic features of chronic lymphocytic leukemia with trisomy 12: a comprehensive review |journal=Haematologica |volume=103 |issue=6 |pages=931–938 |date=June 2018 |pmid=29748447 |pmc=6058775 |doi=10.3324/haematol.2017.186684 |url=}}</ref>
{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"
| valign="top" |
|+
! style="background: #4479BA; width: 600px;" | {{fontcolor|#FFF|'''Differential Diagnosis'''}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|'''Cytogenetics'''}}
! style="background: #4479BA; width: 500px;" | {{fontcolor|#FFF|'''Morphological Features'''}}
! style="background: #4479BA; width: 500px;" | {{fontcolor|#FFF|'''Immunophenotype'''}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''Splenic marginal zone lymphoma'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Deletion 7q, [[trisomy]] 3, [[trisomy]] 12, [[trisomy]] 18,
* Genes mutation [[NOTCH2]], NFkB pathway genes (BIRC3, [[TNFAIP3]], [[MAP3K14]], IKBKB, [[MYD88]] L265P)
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Micronodular pattern of [[lymphocytic]] infiltration of the [[white pulp]]
* Expanded [[mantle]] and [[marginal zone]] component with [[biphasic]] distribution of neoplastic cells
* [[Diffuse]] or [[patchy]] [[red pulp]] involvement penetrating sinuses and cords
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[CD20]]+, [[CD79a]]+[[IgM]]+
* [[CD11c]]+, [[IgD]]+, DBA44+ in more than 50% of the cases
* [[CD5]]+, [[CD25]]+, [[CD103]]+, [[IgG]]+ in less than 50% of the cases
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''Splenic diffuse red pulp lymphoma'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Uncommon: 7q deletion, [[trisomy]] 18, 17p deletion ( [[TP53]])
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* No [[micronodular pattern]]
* [[Effacement]] of [[white pulp]]
* Obliteration of splenic architecture by neoplastic cells
* [[Red pulp]] involvement with infiltration of both cord and sinuses
* Extensive [[intra-sinusoidal]] involvement in the [[bone marrow]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[CD11c]]+,[[CD20]]+, [[CD79a]]+ DBA44+
* [[IgM]]+, [[IgD]]+, [[IgG]]+ in more than 50% of the cases
* [[CD5]]+, [[CD103]]+, [[CD123]]+ in less than 50% of the cases
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''Hairy cell leukemia variant'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Common: Deletion 17p ( [[TP53]]),
* Uncommon: 5q gain, deletion 7q
* Gene mutation: [[MAP2K1]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* More commonly [[sinusoidal]] or [[diffuse]] infiltration of the [[bone marrow]]
* [[Peripheral blood]] picture predominant with [[prolymhocytes]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[CD11c]]+, [[ CD20]]+, [[CD79a]]+, [[CD103]]+, DBA44+
* [[IgG]]+ in more than 50% of the cases
* [[IgD]]+, [[IgM]]+ in less than 50% of the cases
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''Hairy cell leukemia'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Uncommon: 5q gain, 7q deletion
* gene mutation: [[BRAF V600E]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Uniform involvement of [[red pulp]]
* [[white pulp]] reduced in size
* [[patchy]] [[infiltration]] of the [[bone marrow]] by [[hairy cells]].
* increase [[reticulin]] fiber replacing normal [[hematopoietic]] cells resulting in '''Dry tap'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[CD11c]]+, [[CD20]]+, [[CD79a]]+, [[CD25]]+, [[CD103]]+, [[CD123]]+, [[Annexin A1]]+, [[IgM]]+, [[IgD]]+, [[IgG]]+
* [[CD10]]+ in less than 50% of the cases
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''Lymphoplasmacytic lymphoma'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Common: 6q deletion,
* Uncommon: 13q deletion, 7q deletion
* Gene mutation: [[MYD88]] L265P
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Homogeneous]] [[white pulp]] [[infiltration]] without visible [[marginal zone]] and monocytoid cells
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[CD11]]+,[[ CD20]]+, [[CD79a]]+ [[IgM]]+
* [[CD25]]+ in more than 50% of the cases
* [[CD5]]+ in less than 50% of the cases
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''Chronic lymphocytic leukemia'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Chromosomal deletion]] 6q, 7q, 13q, 17p
* [[Trisomy]] 13 less frequently
* Gene mutation: [[TP53]],[[SF3B1]], [[BIRC3]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Small mature [[neoplastic]] [[lymphoid]] cells
* [[Bone marrow]] infiltration pattern may [[diffuse]], [[interstitial]], [[nodular]]
* Intrasinusoidal pattern rarely observed
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[IgM]]+, [[IgD]]+, [[CD5]]+, [[CD23]]+, [[CD43]]+, [[CD79a]]+, [[LEF1]]+
* [[CD20]]+, DBA44+ in less than 50%
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''Follicular lymphoma'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* t(14;18) [[translocation]]
* Gene mutation: BCL2
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[Follicles]] in the [[white pulp]] consisting of centroblasts and centrocytes
* [[Diffuse]] [[red pulp]] [[infiltration]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[IgM]]+, [[IgD]]+, [[CD20]]+, [[CD79a]]+, [[BCL6]]+
* [[CD10]]+ in more than 50%
* [[CD23]]+ in less than 50%
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
'''Mantle cell lymphoma'''
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* t(11;14) [[translocation]]
* [[Gene mutation]]: [[CCND1]]
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* Small neoplastic cells similar to centrocytes
* [[Bone marrow]] [[infiltration]] pattern may be [[interstitial]], [[diffuse]] or [[nodular]]
* Intrasinusoidal pattern rarely observed
| style="padding: 5px 5px; background: #F5F5F5;" align="left" |
* [[IgM]]+, [[IgD]]+, [[CD5]]+, [[CD20]]+, [[CD43]]+, [[CD79a]]+, [[BCL1]]+, [[SOX11]]+
* [[BCL6]]+ and LEF1+ in less than 50% for the cases
|}
 
==References==
==References==
{{reflist|2}}
{{reflist|2}}
{{WH}}
{{WH}}
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{{WS}}
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Latest revision as of 20:49, 28 February 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Muhammad Affan M.D.[2], Sowminya Arikapudi, M.B,B.S. [3]

Overview

Splenic marginal zone lymphoma (SMZL) must be differentiated from other B-cell lymphomas such as chronic lymphocytic leukemia, follicular lymphoma, mantle cell lymphoma and unclassifiable B-cell lymphomas including Hairy cell leukemia variant (HCL-v) and splenic diffuse red pulp small B-cell lymphoma (SDRPL) on the basis of cytogenetics, immunophenotyping and morphological as the treatment for these conditions varies.

Differential Diagnosis

Splenic marginal zone lymphoma(SMZL) must be differentiated from other splenic B-cell lymphomas with the help of clincal, morphological, immunophenotypic and genetic data as the treatment of all these conditions are different from each other.[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][22][27][28]

Differential Diagnosis Cytogenetics Morphological Features Immunophenotype

Splenic marginal zone lymphoma

Splenic diffuse red pulp lymphoma

  • Uncommon: 7q deletion, trisomy 18, 17p deletion ( TP53)

Hairy cell leukemia variant

  • Common: Deletion 17p ( TP53),
  • Uncommon: 5q gain, deletion 7q
  • Gene mutation: MAP2K1

Hairy cell leukemia

  • Uncommon: 5q gain, 7q deletion
  • gene mutation: BRAF V600E

Lymphoplasmacytic lymphoma

  • Common: 6q deletion,
  • Uncommon: 13q deletion, 7q deletion
  • Gene mutation: MYD88 L265P

Chronic lymphocytic leukemia

Follicular lymphoma

Mantle cell lymphoma

References

  1. Matutes E, Oscier D, Montalban C, Berger F, Callet-Bauchu E, Dogan A, Felman P, Franco V, Iannitto E, Mollejo M, Papadaki T, Remstein ED, Salar A, Solé F, Stamatopoulos K, Thieblemont C, Traverse-Glehen A, Wotherspoon A, Coiffier B, Piris MA (March 2008). "Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria". Leukemia. 22 (3): 487–95. doi:10.1038/sj.leu.2405068. PMID 18094718.
  2. Salido M, Baró C, Oscier D, Stamatopoulos K, Dierlamm J, Matutes E, Traverse-Glehen A, Berger F, Felman P, Thieblemont C, Gesk S, Athanasiadou A, Davis Z, Gardiner A, Milla F, Ferrer A, Mollejo M, Calasanz MJ, Florensa L, Espinet B, Luño E, Wlodarska I, Verhoef G, García-Granero M, Salar A, Papadaki T, Serrano S, Piris MA, Solé F (September 2010). "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". Blood. 116 (9): 1479–88. doi:10.1182/blood-2010-02-267476. PMID 20479288.
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