Difference between revisions of "Small cell lymphomas"

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Small cell lymphomas (view source)

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hive off hairy cell leukemia to its own page
(hive off hairy cell leukemia to its own page)
 
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! Other
! Other
|-
|-
| Follicular lymphoma
| [[Follicular lymphoma]]
| Follicle
| Follicle
| Small, centrocytes, centroblasts
| Small, centrocytes, centroblasts
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| 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) 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>)
| 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>)
| aggressive, poor prognosis<ref name=pmid10583923/>
| 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]]
|-
|-
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| location ?
| location ?
| small
| small
| CD5+, CD23+, CD43+, cyclin D1-
| CD5+, [[CD23]]+, CD43+, cyclin D1-
| trisomy 12; deletions of 11q, 13q, 17p<ref>{{Ref PCPBoD8|318}}</ref>
| trisomy 12; deletions of 11q, 13q, 17p<ref>{{Ref PCPBoD8|318}}</ref>
| good prognosis / indolent course
| good prognosis / indolent course
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|}
|}


Medium and large cell lymphomas:
Common medium and large cell lymphomas:
{| class="wikitable"
{| class="wikitable"
! Name
! Name
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| [[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
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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 (lymph node):
*Abundant abnormally-shaped 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).
 
Note:
*The intrafollicular component of the lymph node is compressed - follicles are often described as "kissing", as they nearly touch.
*In [[bone marrow]] specimens the neoplastic cells classically have a paratrabecular arrangement,<ref name=pmid17284114>{{Cite journal  | last1 = Iancu | first1 = D. | last2 = Hao | first2 = S. | last3 = Lin | first3 = P. | last4 = Anderson | first4 = SK. | last5 = Jorgensen | first5 = JL. | last6 = McLaughlin | first6 = P. | last7 = Medeiros | first7 = LJ. | title = Follicular lymphoma in staging bone marrow specimens: correlation of histologic findings with the results of flow cytometry immunophenotypic analysis. | journal = Arch Pathol Lab Med | volume = 131 | issue = 2 | pages = 282-7 | month = Feb | year = 2007 | doi = 10.1043/1543-2165(2007)131[282:FLISBM]2.0.CO;2 | PMID = 17284114 }}</ref> i.e. the lymphoma cells are found adjacent to the bone spicules.
 
Image:
*[http://www.flickr.com/photos/euthman/3311641845/ Follicular lymphoma - bone marrow (flickr.com)].
====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==
*Abbreviated ''MCL''.
*Abbreviated ''MCL''.
===General===
{{Main|Mantle cell lymphoma}}
*Relatively aggressive - guarded 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>
 
===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]].
 
Images:
*[http://path.upmc.edu/cases/case441.html MCL - several images (upmc.edu)].
 
===IHC===
*CD5 +ve.
**Negative in case reports.<ref>URL: [http://path.upmc.edu/cases/case308/dx.html http://path.upmc.edu/cases/case308/dx.html]. Accessed on: 14 January 2012.</ref>
*CD43 +ve.
*Cyclin D1 +ve.
**[[AKA]] BCL1, AKA CCND1.<ref name=omim168461>{{OMIM|168461}}</ref>
 
Others:
*CD23 -ve.
**Positive in CLL.
 
===Molecular===
*t(11;14)(q13;q32) / IGH-CCND1.<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''.
*Arise in the context chronic infections, e.g. [[Sjögren disease]] ([[salivary gland]]), [[Hashimoto thyroiditis]] ([[thyroid gland]]), [[Helicobacter pylori gastritis]] ([[stomach]]).<ref name=Ref_PCPBoD8_326>{{Ref PCPBoD8|326}}</ref>
 
===Classification===
*Comes in three different flavours:
*#Extranodal marginal zone lymphoma.
*#*If in ''mucosa-associated lymphoid tissue'' known as a ''MALT lymphoma'', [[AKA]] ''MALToma''.
*#Splenic marginal zone lymphoma (SMZL).
*#Nodal marginal zone lymphoma (NMZL).
 
===Microscopic===
Features:
*Small (lymphoid) cells that may be plasma cell-like (plasmacytoid):<ref>URL: [http://surgpathcriteria.stanford.edu/bcell/marginalnodal/printable.html http://surgpathcriteria.stanford.edu/bcell/marginalnodal/printable.html]. Accessed on: 6 March 2012.</ref>
**+/-Clockface nucleus.
**+/-Eccentric nucleus.
*+/-"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>
**Features:
**# Cluster of lymphocytes - three cells or more - '''key feature'''.
**#* 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:
*[[WC]]:
**[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)].
*www:
**[http://path.upmc.edu/cases/case405.html MALT lymphoma - several images (upmc.edu)].
 
===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.
===Molecular===
There are several associated with MALT lymphoma:<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>
*t(11;18)(q21;q21) / API2‐MALT1<ref name=Ref_PCPBoD8_170>{{Ref PCPBoD8|170}}</ref> - most common translocation in MALT lymphoma.<ref name=pmid12406890>{{Cite journal  | last1 = Streubel | first1 = B. | last2 = Lamprecht | first2 = A. | last3 = Dierlamm | first3 = J. | last4 = Cerroni | first4 = L. | last5 = Stolte | first5 = M. | last6 = Ott | first6 = G. | last7 = Raderer | first7 = M. | last8 = Chott | first8 = A. | title = T(14;18)(q32;q21) involving IGH and MALT1 is a frequent chromosomal aberration in MALT lymphoma. | journal = Blood | volume = 101 | issue = 6 | pages = 2335-9 | month = Mar | year = 2003 | doi = 10.1182/blood-2002-09-2963 | PMID = 12406890 }}
</ref>
*t(14;18)(q32;q21) / IGH‐MALT1.
**Should not be confused with t(14;18) seen in [[follicular lymphoma]] between IGH-BCL2.<ref name=pmid18359244/>
*t(1;14)(p22;q32) / IGH‐BCL10.


The MALT1 associated [[translocations]] can be assessed with an [[ISH]] break apart probe for MALT1.
Classification:
#Extranodal marginal zone lymphoma.
#*If in ''mucosa-associated lymphoid tissue'' known as a ''MALT lymphoma'', [[AKA]] ''MALToma''.
#Splenic marginal zone lymphoma (SMZL).
#Nodal marginal zone lymphoma (NMZL).


==Hairy cell leukemia==
==Hairy cell leukemia==
*Abbreviated ''HCL''.
See [[Hairy cell leukemia]]
===General===
*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.
 
===Gross===
Features:<ref name=Ref_PCPBoD8_326>{{Ref PCPBoD8|326}}</ref>
*Huge beefy red [[spleen]].
**Red as white pulp obliterated.
 
===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'''.
 
DDx:
*[[Hepatosplenic T-cell lymphoma]].
 
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Hairy_cell_leukemia.jpg HCL - blood film (WC)].
**[http://commons.wikimedia.org/wiki/File:Hairy_cell_leukemia_-_very_high_mag.jpg HCL - very high mag. (WC)].
*www:
**[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://path.upmc.edu/cases/case240.html HCL - several images (upmc.edu)].
**[http://path.upmc.edu/cases/case691.html HCL - another case with several images (upmc.edu)].
**[http://webpathology.com/image.asp?case=380&n=3 HCL in the spleen (webpathology.com)].
 
===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==
*Abbreviated ''CLL'' and ''SLL''.
{{Main|B cell small lymphocytic lymphoma/chronic lymphocytic leukemia}}
===General===
*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)].
*[http://path.upmc.edu/cases/case296.html SLL in the parotid - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case446.html CLL with Richter transformation - several images (upmc.edu)].
 
===IHC===
*CD20 +ve.
*CD5 +ve.
*CD23 +ve -- occasionally negative.<ref>URL: [http://path.upmc.edu/cases/case296/dx.html http://path.upmc.edu/cases/case296/dx.html]. Accessed on: 14 January 2012.</ref>
*CD43 +ve.
 
Others:
*Cyclin D1 -ve.
 
===Molecular===
*Lacks t(11;14) seen in [[mantle cell lymphoma]].


==Precursor B-cell lymphoblastic lymphoma/leukemia ==
==Precursor B-cell lymphoblastic lymphoma/leukemia ==
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[[Category:Haematopathology]]
[[Category:Haematopathology]]
[[Category:Small cell lymphomas]]
Mark
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