Difference between revisions of "Small cell lymphomas"

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Small cell lymphomas (view source)

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'''Small cell [[lymphoma]]s''' are a collection of commonly seen lymphomas that have a near-identical histomorphologic appearance.
The '''small cell lymphomas''' are a collection of commonly seen [[lymphoma]]s that have a near-identical histomorphologic appearance.


The group includes:
The group includes:
#Small lymphocytic lymphoma/chronic lymphocytic leukemia.
#Small lymphocytic lymphoma/[[chronic lymphocytic leukemia]].
#Follicular lymphoma.
#[[Follicular lymphoma]].
#Mantle cell lymphoma.
#[[Mantle cell lymphoma]].
#Marginal zone lymphoma (includes ''MALT lymphoma'').
#[[Marginal zone lymphoma]] (includes ''[[MALT lymphoma]]'').
#Hairy cell leukemia.
#[[Hairy cell leukemia]].
#Immunoproliferative small intestinal disease (IPSID).<ref name=pmid15542584>{{cite journal |author=Al-Saleem T, Al-Mondhiry H |title=Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms |journal=Blood |volume=105 |issue=6 |pages=2274–80 |year=2005 |month=March |pmid=15542584 |doi=10.1182/blood-2004-07-2755 |url=http://bloodjournal.hematologylibrary.org/cgi/content/long/105/6/2274}}></ref>
#Immunoproliferative small intestinal disease (IPSID).<ref name=pmid15542584>{{cite journal |author=Al-Saleem T, Al-Mondhiry H |title=Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms |journal=Blood |volume=105 |issue=6 |pages=2274–80 |year=2005 |month=March |pmid=15542584 |doi=10.1182/blood-2004-07-2755 |url=http://bloodjournal.hematologylibrary.org/cgi/content/long/105/6/2274}}></ref>


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! Other
! Other
|-
|-
| Follicular lymphoma
| [[Follicular lymphoma]]
| Follicle
| Follicle
| Small, centrocytes, centroblasts
| Small, centrocytes, centroblasts
| CD10+, BCL6+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| CD10+, BCL6+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| t(14,18)
| t(14;18)(q32;q21) IGH/BCL2<ref name=pmid21192062>{{Cite journal  | last1 = Yanai | first1 = S. | last2 = Nakamura | first2 = S. | last3 = Takeshita | first3 = M. | last4 = Fujita | first4 = K. | last5 = Hirahashi | first5 = M. | last6 = Kawasaki | first6 = K. | last7 = Kurahara | first7 = K. | last8 = Sakai | first8 = Y. | last9 = Matsumoto | first9 = T. | title = Translocation t(14;18)/IGH-BCL2 in gastrointestinal follicular lymphoma: correlation with clinicopathologic features in 48 patients. | journal = Cancer | volume =  | issue =  | pages =  | month = Dec | year = 2010 | doi = 10.1002/cncr.25811 | PMID = 21192062 }}</ref>
| may transform into DLBCL
| may transform into DLBCL
| cery common
| very common
|-
|-
| Mantle cell lymphoma
| Mantle cell lymphoma
| Mantle zone
| mantle zone
| Small
| small
| CD5+, CD23-, CD43+, cyclin D1+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| CD5+, [[CD23]]-, CD43+, cyclin D1+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| t(11;14)(q13;q32)<ref>URL: [http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html]. Accessed on: 10 August 2010.</ref>
| t(11;14)(q13;q32) BCL1/IGH<ref>URL: [http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html]. Accessed on: 10 August 2010.</ref> (also IGH/BCL1<ref>URL: [http://www.wipo.int/patentscope/search/en/WO2010059499 http://www.wipo.int/patentscope/search/en/WO2010059499]. Accessed on: 26 May 2011.</ref>)
| Clinical ?
| aggressive, poor prognosis<ref name=pmid10583923>{{Cite journal  | last1 = Hankin | first1 = RC. | last2 = Hunter | first2 = SV. | title = Mantle cell lymphoma. | journal = Arch Pathol Lab Med | volume = 123 | issue = 12 | pages = 1182-8 | month = Dec | year = 1999 | doi = 10.1043/0003-9985(1999)1231182:MCL2.0.CO;2 | PMID = 10583923 }}</ref>
| DDx: [[Castleman disease]]
| DDx: [[Castleman disease]]
|-
|-
| Marginal zone lymphoma (includes MALT)
| Marginal zone lymphoma (includes MALT)
| Marginal zone
| [[lymph node|marginal zone]], [[spleen]], GI tract
| Small
| small
| CD21+, CD11c+, CD5-, CD23-<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| CD21+, CD11c+, CD5-, CD23-<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| Translocations
| t(11;18)(q21;q21) / API2‐MALT1, t(14;18)(q32;q21) / IGH‐MALT1, t(1;14)(p22;q32) / IGH‐BCL10<ref name=pmid16950858>{{cite journal |author=Bacon CM, Du MQ, Dogan A |title=Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists |journal=J. Clin. Pathol. |volume=60 |issue=4 |pages=361–72 |year=2007 |month=April |pmid=16950858 |pmc=2001121 |doi=10.1136/jcp.2005.031146 |url=}}</ref>
| classical GI lymphomas
 
| Other
| classical GI lymphoma
| subtypes: extranodal marginal zone lymphoma ([[AKA]] MALT lymphoma), SMZL, nodal marginal zone lymphoma
|-
|-
| Precursor B cell lymphoblastic lymphoma/leukemia
| Precursor B cell lymphoblastic lymphoma/leukemia
| Location ?
| location ?
| Small
| small
| CD10+, CD5-, TdT+, CD99+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| CD10+, CD5-, TdT+, CD99+<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
| Translocations ?
| t(9;22), others
| aggressive (?)
| good prognosis (?)
| Other ?
| other ?
|-
|-
| B cell small lymphocytic lymphoma /<br>chronic lymphocytic leukemia
| B cell small lymphocytic lymphoma /<br>chronic lymphocytic leukemia
| Location ?
| location ?
| Small
| small
| CD5+, CD23+, CD43+, cyclin D1-
| CD5+, [[CD23]]+, CD43+, cyclin D1-
| Translocations ?
| trisomy 12; deletions of 11q, 13q, 17p<ref>{{Ref PCPBoD8|318}}</ref>
| good prognosis / indolent course
| good prognosis / indolent course
| Other ?
| other ?
|- <!--
|- <!--
| Name/entity ?
| Name/entity ?
| Location ?
| location ?
| Size of cells ?
| size of cells ?
| IHC ?
| IHC ?
| Translocations ?
| translocations ?
| Clinical ?
| clinical ?
| Other ? -->
| other ? -->
|}
|}


Medium and large cell lymphomas:
Common medium and large cell lymphomas:
{| class="wikitable"
{| class="wikitable"
! Name
! Name
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! IHC
! IHC
! Translocations
! Translocations
! Clinical
! Clinical  
! Other
! Other
|-
|-
| Burkitt's lymphoma
| [[Burkitt's lymphoma]]
| Follicle
| follicle
| Large cells
| large cells
| CD10, BCL6
| CD10, BCL6
| t(8;14) (q24;q32)  
| t(8;14) (q24;q32)  
| Rapid growth
| rapid growth
| "Starry sky"
| "starry sky"
|-
|-
| Diffuse large B cell lymphoma
| [[Diffuse large B cell lymphoma]]
| Follicle (?)
| follicle (?)
| Large 4-5X of lymphocyte
| large 4-5X of lymphocyte
| MIB-1 >40%
| MIB1 >40%
| none/like follicular l.
| none/like follicular l.
| Poor prognosis
| poor prognosis
| Common among lymphomas
| common among lymphomas
<!--
<!--
|-
|-
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==Follicular lymphoma==
==Follicular lymphoma==
===General===
*Abbreviated ''FL''.
*A very common type of lymphoma.
{{Main|Follicular lymphoma}}
*Expresses BCL2,<ref name=pmid18359244>{{cite journal |author=Vitolo U, Ferreri AJ, Montoto S |title=Follicular lymphomas |journal=Crit. Rev. Oncol. Hematol. |volume=66 |issue=3 |pages=248–61 |year=2008 |month=June |pmid=18359244 |doi=10.1016/j.critrevonc.2008.01.014 |url=}}</ref> like many other small cell lymphomas.
 
===Microscopic===
Features:
*Abundant abnormal lymphoid follicles - '''key feature''' - including some of the following:
**Non-polarized mantle zone (normal mantle zone is usu. thicker at capsular aspect).
**Non-polarized germinal center (normal germinal center has dark & light area).
**Loss of tingible body macrophages.
**Sinuses effaced (lost).
 
====Grading====
*Grade 1-2: <= 22 centroblasts / HPF; where 1 HPF ~= 0.2376 mm^2 (22 mm eye piece @ 40X objective).
*Grade 3A: >22 centroblasts / HPF; where 1 HPF ~= 0.2376 mm^2 (22 mm eye piece @ 40X objective).
*Grade 3B: only centroblasts (within a nodular architecture).
 
Notes:
*Significant interobserver variability.<ref>DG. 17 August 2010.</ref>
*Grade 1 & Grade 2 lumped together.
*One should evaluate 10 HPFs.
*''Only'' centroblasts ''without'' a nodular architecture is ''[[Diffuse large B cell lymphoma]]'' ([[DLBCL]]).
 
The usual cut points mentioned by people with [[HPFitis]] are:<ref name=Ref_Sternberg4_813>{{Ref Sternberg4|813}}</ref>
*Grade 1: 0-5 centroblasts / HPF.
*Grade 2: 5-15 centroblasts / HPF.
*Grade 3: >15 centroblasts / HPF.
 
===IHC===
Features:<ref name=pmid18359244/>
*CD10 +ve.
*BCL6 +ve.
 
Others:
*CD5 -ve.
**+ve in mantle cell lymphoma.
*CD23 -ve/+ve.
**+ve in CLL.
*CD43 -ve.
**+ve in mantle cell lymphoma, marginal zone lymphoma.
*CD11c -ve -- flow cytometry only.
 
===Molecular===
*t(14;18)(q32;q21)/IGH-BCL2 in 70-95% of cases.<ref name=pmid18359244/>
**Should not be confused with t(14;18)(q32;q21)/IGH-MALT1 seen in MALT lymphomas.<ref name=pmid16950858>{{cite journal |author=Bacon CM, Du MQ, Dogan A |title=Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists |journal=J. Clin. Pathol. |volume=60 |issue=4 |pages=361–72 |year=2007 |month=April |pmid=16950858 |pmc=2001121 |doi=10.1136/jcp.2005.031146 |url=}}</ref>


==Mantle cell lymphoma==
==Mantle cell lymphoma==
===General===
*Abbreviated ''MCL''.
*Comes in different flavours.
{{Main|Mantle cell lymphoma}}
 
===Microscopic===
Features:<ref>DG. 17 August 2010.</ref>
*Small monomorphic (uniform size, shape and staining) lymphoid population.
*Abundant mitoses.
*Scattered epithelioid histiocytes (should not be confused with tingible-body macrophages).
*Sclerosed blood vessels.
 
DDx:
*Other small cell lymphomas, esp. marginal zone lymphoma.
*Burkitt's lymphoma.
 
===IHC===
*CD5 +ve.
*CD43 +ve.
*Cyclin D1 +ve.
**[[AKA]] Bcl-1.
 
Others:
*CD23 -ve.
**Positive in CLL.
 
===Molecular===
*t(11;14)(q13;q32).<ref>URL: [http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html]. Accessed on: 10 August 2010.</ref>


==Marginal zone lymphoma==
==Marginal zone lymphoma==
===General===
{{Main|Marginal zone lymphoma}}
*Abbreviated as ''MZL''.
 
===Classification===
*Comes in three different flavours:
**Extranodal marginal zone lymphoma (AKA ''MALT lymphoma'' = mucosa-associated lymphoid tissue lymphoma).
**Splenic marginal zone lymphoma.
**Nodal marginal zone lymphoma (NMZL).


===Microscopic===
Classification:
Features:
#Extranodal marginal zone lymphoma.
*"Lymphoepithelial lesion" - gastric crypts invaded by a monomorphous population of lymphocytes.<ref name=pmid1452124>{{Cite journal  | last1 = Papadaki | first1 = L. | last2 = Wotherspoon | first2 = AC. | last3 = Isaacson | first3 = PG. | title = The lymphoepithelial lesion of gastric low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT): an ultrastructural study. | journal = Histopathology | volume = 21 | issue = 5 | pages = 415-21 | month = Nov | year = 1992 | doi =  | PMID = 1452124 }}</ref>
#*If in ''mucosa-associated lymphoid tissue'' known as a ''MALT lymphoma'', [[AKA]] ''MALToma''.
**Features:
#Splenic marginal zone lymphoma (SMZL).
**# Cluster of lymphocytes - three cells or more - '''key feature'''.
#Nodal marginal zone lymphoma (NMZL).
**#* Single lymphocytes don't count.
**# Clearing around the lymphocyte cluster.
**Not specific for MALT lymphoma, i.e. may be seen in other types of lymphoma.<ref>DB. 6 August 2010.</ref>
 
Images:
*[http://commons.wikimedia.org/wiki/File:Gastrointestinal_lymphoepithelial_lesion_-_very_high_mag.jpg Lymphoepithelial lesion - very high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Gastrointestinal_lymphoepithelial_lesion_-_intermed_mag.jpg Lymphoepithelial lesion - intermed. mag. (WC)].
 
===IHC===
Features:<ref>{{Ref Lester|95}}</ref>
*CD20 +ve.
*BCL2 +ve.
*CD21 +ve.
*CD11c +ve (flow cytometry or laser scanning cytometry - only; not available for paraffin).
*CD43 +ve/-ve.
**Typically positive in ''[[mantle cell lymphoma]].
 
Others:
*CD5 -ve.
*CD10 -ve.
*CD23 -ve.


==Hairy cell leukemia==
==Hairy cell leukemia==
===General===
See [[Hairy cell leukemia]]
*Name comes from appearance on blood smear - cell hairy.
 
Clinical:<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022]. Accessed on: 20 August 2010.</ref>
*Pancytopenia.
*Splenic enlargement.
*No lymphadenopathy.
*Good prognosis (with treatment), though (likely) not curable.
 
===Microscopic===
Features:<ref>URL: [http://emedicine.medscape.com/article/200580-diagnosis http://emedicine.medscape.com/article/200580-diagnosis]. Accessed on: 18 August 2010.</ref>
*Small cells (10-20 micrometers) with "Fried egg"-like appearance:
**Well-demarcated fuzzy cell borders,
**Clear/whispy cytoplasm and,
**Central round nucleus.
***Peri-nuclear clearing ("water-clear rim"<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022]. Accessed on: 20 August 2010.</ref>) -- '''key feature'''.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Hairy_cell_leukemia.jpg HCL - blood film (WC)].
*[http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=figure&id=A34027 HCL - bone marrow (nlm.nih.gov)] from [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 Holland-Frei Cancer Medicine (nlm.nih.gov)].
*[http://commons.wikimedia.org/wiki/File:Hairy_cell_leukemia_-_very_high_mag.jpg HCL - very high mag. (WC)].
 
===IHC===
Features:<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029]. Accessed on: 20 August 2010.</ref>
*CD20 +ve, CD25 +ve, CD103 +ve.
*CD5 -ve.
 
Flow cytometry:
*CD19 +ve, CD11c +ve, FMC7 +ve.


==B cell small lymphocytic lymphoma/chronic lymphocytic leukemia==
==B cell small lymphocytic lymphoma/chronic lymphocytic leukemia==
===General===
{{Main|B cell small lymphocytic lymphoma/chronic lymphocytic leukemia}}
*Abbreviated ''CLL'' and ''SLL''.
*Very common.
*Good prognosis.
 
====Richter's transformation====
*CLL/SLL may under go a ''Richter's transformation'' into a high-grade non-Hodgkin's lymphoma (NHL), e.g. [[DLBCL]]:<ref name=pmid16616072>{{cite journal |author=Tsimberidou AM, Keating MJ |title=Richter's transformation in chronic lymphocytic leukemia |journal=Semin. Oncol. |volume=33 |issue=2 |pages=250–6 |year=2006 |month=April |pmid=16616072 |doi=10.1053/j.seminoncol.2006.01.016 |url=}}</ref>
**Incidence of transformation <5%.
**Prognosis < 1 year.
 
===Microscopic===
Features in a lymph node:<ref>DG. 17 August 2010.</ref>
*Mixed population of lymphoid cells with "proliferation centers" - '''key feature''':
*#Larger cells (~ 1.5x the size of resting lymphocyte ~ 12-15 micrometers):
*#*Nucleoli.
*#*Form (nodular) collections.
*#Small dark cells (~ size of resting lymphocyte ~ 8-10 micrometers):
*#*Predominant population.
*#*Lack nucleolus.
 
Images:
*[http://www.pathologyatlas.ro/chronic-lymphoid-leukemia-liver.php CLL in the liver (pathologyatlas.ro)].


==Precursor B-cell lymphoblastic lymphoma/leukemia ==
==Precursor B-cell lymphoblastic lymphoma/leukemia ==
===General===
===General===
*Prognosis poor. (???)
*Good prognosis.
*Paediatric - usu. <6 years old.


===Microscopic===
===Microscopic===
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*CD10 +ve, TdT +ve, CD99 +ve.
*CD10 +ve, TdT +ve, CD99 +ve.
*CD5 -ve.
*CD5 -ve.
===Molecular===
Subclassification based on molecular abnormalities (translocations, rearrangements):<ref name=pmid15559730>{{cite journal |author=Randolph TR |title=Advances in acute lymphoblastic leukemia |journal=Clin Lab Sci |volume=17 |issue=4 |pages=235–45 |year=2004 |pmid=15559730 |url=http://findarticles.com/p/articles/mi_qa3890/is_200410/ai_n9429273/pg_2}}</ref>
* t(9;22) / BCR-ABL.
* t(1;19) / E2A-PBX1.
* t(12;21) / ETV-CBFalpha.
* MLL rearrangement.


==Precursor T-cell lymphoblastic lymphoma/leukemia ==
==Precursor T-cell lymphoblastic lymphoma/leukemia ==
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*[[Haematopathology]].
*[[Haematopathology]].
*[[Lymph node pathology]].
*[[Lymph node pathology]].
*[[Spleen]].


==References==
==References==
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[[Category:Haematopathology]]
[[Category:Haematopathology]]
[[Category:Small cell lymphomas]]
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