Difference between revisions of "Small cell lymphomas"

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'''Small cell lymphomas''' 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.
#[[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>
| Clinical ?
| may transform into DLBCL
| Other ?
| 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>
| Other ?
| DDx: [[Castleman disease]]
|-
|-
| Marginal zone lymphoma (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>
| Clinical
 
| Other
| classical GI lymphoma
| subtypes: extranodal marginal zone lymphoma ([[AKA]] MALT lymphoma), SMZL, nodal marginal zone lymphoma
|-
|-
| Precursor 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
| Clinical ?
| good prognosis (?)
| Other ?
| other ?
|-
| Small lymphocytic lymphoma/chronic lymphocytic leukemia
| Location ?
| Small
| CD5+, CD23+, CD43+, cyclin D1-
| Translocations ?
| Good prognosis
| Other ?
<!--
|-
|-
| B cell small lymphocytic lymphoma /<br>chronic lymphocytic leukemia
| location ?
| small
| CD5+, [[CD23]]+, CD43+, cyclin D1-
| trisomy 12; deletions of 11q, 13q, 17p<ref>{{Ref PCPBoD8|318}}</ref>
| good prognosis / indolent course
| 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===
==Mantle cell lymphoma==
Features:
*Abbreviated ''MCL''.
*Abundant abnormal lymphoid follicles - '''key feature''' - including some of the following:
{{Main|Mantle cell lymphoma}}
**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====
==Marginal zone lymphoma==
*Grade 1-2: <= 22 centroblasts / HPF; where 1 HPF ~= 0.2376 mm^2 (22 mm eye piece @ 40X objective).
{{Main|Marginal zone lymphoma}}
*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:
Classification:
*Significant interobserver variability.<ref>DG. 17 August 2010.</ref>
#Extranodal marginal zone lymphoma.
*Grade 1 & Grade 2 lumped together.
#*If in ''mucosa-associated lymphoid tissue'' known as a ''MALT lymphoma'', [[AKA]] ''MALToma''.
*One should evaluate 10 HPFs.
#Splenic marginal zone lymphoma (SMZL).
*''Only'' centroblasts ''without'' a nodular architecture is ''[[Diffuse large B cell lymphoma]]'' ([[DLBCL]]).
#Nodal marginal zone lymphoma (NMZL).


The usual cut points mentioned by people with [[HPFitis]] are:<ref name=Ref_Sternberg4_813>{{Ref Sternberg4|813}}</ref>
==Hairy cell leukemia==
*Grade 1: 0-5 centroblasts / HPF.
See [[Hairy cell leukemia]]
*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===
==B cell small lymphocytic lymphoma/chronic lymphocytic leukemia==
*t(14;18)(q32;q21)/IGH-BCL2 in 70-95% of cases.<ref name=pmid18359244/>
{{Main|B cell small lymphocytic lymphoma/chronic lymphocytic leukemia}}
**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==
==Precursor B-cell lymphoblastic lymphoma/leukemia ==
===General===
===General===
*Comes in different flavours.
*Good prognosis.
*Paediatric - usu. <6 years old.


===Microscopic===
===Microscopic===
Features:<ref>DG. 17 August 2010.</ref>
Features:<ref>DG. 17 August 2010.</ref>
*Small monomorphic lymphoid population.
*High mitotic rate.
*Abundant mitoses.
*"Starry sky" pattern.
*Scatter epithelioid histiocytes (should not be confused with tingible-body macrophages).
*Small nucleoli.
*Sclerosed blood vessels.
 
DDx:
*Other small cell lymphomas, esp. marginal zone lymphoma.
*Burkitt's lymphoma.


===IHC===
===IHC===
*CD5 +ve.
Features:<ref name=Ref_Lester95>{{Ref Lester|95}}</ref>
*CD43 +ve.
*CD10 +ve, TdT +ve, CD99 +ve.
*Cyclin D1 +ve.
*CD5 -ve.
 
Others:
*CD23 -ve.
**Positive in CLL.


===Molecular===
===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>
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.


==MALT lymphoma/Marginal zone lymphoma==
==Precursor T-cell lymphoblastic lymphoma/leukemia ==
===General===
===General===
*Abbreviated as ''MZL''.
*Prognosis poor. (???)
 
===Classification===
*Comes in different flavours:
**Extranodal marginal zone lymphoma.
**Splenic marginal zone lymphoma.


===Microscopic===
===Microscopic===
Features:
Features:
*See ''histomorphology of small cell lymphomas''.
*Small lymphoid cells. (???)
*"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>


==Hairy cell leukemia==
===IHC===
===General===
Features:<ref name=Ref_Lester97>{{Ref Lester|97}}</ref>
*Name comes from appearance on blood smear - cell hairy.
*TdT +ve, CD34 +ve, CD99 +ve, CD1a +ve/-ve.
 
*TIA1 -ve.
===Microscopic===
Features:
*See ''histomorphology of small cell lymphomas''.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Hairy_cell_leukemia.jpg HCL - blood film (WC)].
 
==Small lymphocytic lymphoma/chronic lymphocytic leukemia==
===General===
*Very common.
*Good prognosis.
 
===Microscopic===
Features:
*See ''histomorphology of small cell lymphomas''.


==See also==
==See also==
*[[Lymphoma]].
*[[Haematopathology]].
*[[Haematopathology]].
*[[Lymph node pathology]].
*[[Lymph node pathology]].
*[[Spleen]].


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

Latest revision as of 20:50, 16 June 2018

The small cell lymphomas are a collection of commonly seen lymphomas that have a near-identical histomorphologic appearance.

The group includes:

  1. Small lymphocytic lymphoma/chronic lymphocytic leukemia.
  2. Follicular lymphoma.
  3. Mantle cell lymphoma.
  4. Marginal zone lymphoma (includes MALT lymphoma).
  5. Hairy cell leukemia.
  6. Immunoproliferative small intestinal disease (IPSID).[1]

Table of B-cell lymphoma

Small cell lymphomas:

Name Location Size of cells IHC Translocations Clinical Other
Follicular lymphoma Follicle Small, centrocytes, centroblasts CD10+, BCL6+[2] t(14;18)(q32;q21) IGH/BCL2[3] may transform into DLBCL very common
Mantle cell lymphoma mantle zone small CD5+, CD23-, CD43+, cyclin D1+[2] t(11;14)(q13;q32) BCL1/IGH[4] (also IGH/BCL1[5]) aggressive, poor prognosis[6] DDx: Castleman disease
Marginal zone lymphoma (includes MALT) marginal zone, spleen, GI tract small CD21+, CD11c+, CD5-, CD23-[2] t(11;18)(q21;q21) / API2‐MALT1, t(14;18)(q32;q21) / IGH‐MALT1, t(1;14)(p22;q32) / IGH‐BCL10[7] classical GI lymphoma subtypes: extranodal marginal zone lymphoma (AKA MALT lymphoma), SMZL, nodal marginal zone lymphoma
Precursor B cell lymphoblastic lymphoma/leukemia location ? small CD10+, CD5-, TdT+, CD99+[2] t(9;22), others good prognosis (?) other ?
B cell small lymphocytic lymphoma /
chronic lymphocytic leukemia 		location ? small CD5+, CD23+, CD43+, cyclin D1- trisomy 12; deletions of 11q, 13q, 17p[8] good prognosis / indolent course other ?

Common medium and large cell lymphomas:

Name Location Size of cells IHC Translocations Clinical Other
Burkitt's lymphoma follicle large cells CD10, BCL6 t(8;14) (q24;q32) rapid growth "starry sky"
Diffuse large B cell lymphoma follicle (?) large 4-5X of lymphocyte MIB1 >40% none/like follicular l. poor prognosis common among lymphomas

Follicular lymphoma

  • Abbreviated FL.
Main article: Follicular lymphoma

Mantle cell lymphoma

  • Abbreviated MCL.
Main article: Mantle cell lymphoma

Marginal zone lymphoma

Main article: Marginal zone lymphoma

Classification:

  1. Extranodal marginal zone lymphoma.
    • If in mucosa-associated lymphoid tissue known as a MALT lymphoma, AKA MALToma.
  2. Splenic marginal zone lymphoma (SMZL).
  3. Nodal marginal zone lymphoma (NMZL).

Hairy cell leukemia

See Hairy cell leukemia

B cell small lymphocytic lymphoma/chronic lymphocytic leukemia

Main article: B cell small lymphocytic lymphoma/chronic lymphocytic leukemia

Precursor B-cell lymphoblastic lymphoma/leukemia

General

  • Good prognosis.
  • Paediatric - usu. <6 years old.

Microscopic

Features:[9]

  • High mitotic rate.
  • "Starry sky" pattern.
  • Small nucleoli.

IHC

Features:[2]

  • CD10 +ve, TdT +ve, CD99 +ve.
  • CD5 -ve.

Molecular

Subclassification based on molecular abnormalities (translocations, rearrangements):[10]

  • t(9;22) / BCR-ABL.
  • t(1;19) / E2A-PBX1.
  • t(12;21) / ETV-CBFalpha.
  • MLL rearrangement.

Precursor T-cell lymphoblastic lymphoma/leukemia

General

  • Prognosis poor. (???)

Microscopic

Features:

  • Small lymphoid cells. (???)

IHC

Features:[11]

  • TdT +ve, CD34 +ve, CD99 +ve, CD1a +ve/-ve.
  • TIA1 -ve.

See also

References

  1. ↑ Al-Saleem T, Al-Mondhiry H (March 2005). "Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms". Blood 105 (6): 2274–80. doi:10.1182/blood-2004-07-2755. PMID 15542584. http://bloodjournal.hematologylibrary.org/cgi/content/long/105/6/2274.>
  2. ↑ 2.0 2.1 2.2 2.3 2.4 Lester, Susan Carole (2005). Manual of Surgical Pathology (2nd ed.). Saunders. pp. 95. ISBN 978-0443066450.
  3. ↑ Yanai, S.; Nakamura, S.; Takeshita, M.; Fujita, K.; Hirahashi, M.; Kawasaki, K.; Kurahara, K.; Sakai, Y. et al. (Dec 2010). "Translocation t(14;18)/IGH-BCL2 in gastrointestinal follicular lymphoma: correlation with clinicopathologic features in 48 patients.". Cancer. doi:10.1002/cncr.25811. PMID 21192062.
  4. ↑ URL: http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html. Accessed on: 10 August 2010.
  5. ↑ URL: http://www.wipo.int/patentscope/search/en/WO2010059499. Accessed on: 26 May 2011.
  6. ↑ Hankin, RC.; Hunter, SV. (Dec 1999). "Mantle cell lymphoma.". Arch Pathol Lab Med 123 (12): 1182-8. doi:10.1043/0003-9985(1999)1231182:MCL2.0.CO;2. PMID 10583923.
  7. ↑ Bacon CM, Du MQ, Dogan A (April 2007). "Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists". J. Clin. Pathol. 60 (4): 361–72. doi:10.1136/jcp.2005.031146. PMC 2001121. PMID 16950858. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2001121/.
  8. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 318. ISBN 978-1416054542.
  9. ↑ DG. 17 August 2010.
  10. ↑ Randolph TR (2004). "Advances in acute lymphoblastic leukemia". Clin Lab Sci 17 (4): 235–45. PMID 15559730. http://findarticles.com/p/articles/mi_qa3890/is_200410/ai_n9429273/pg_2.
  11. ↑ Lester, Susan Carole (2005). Manual of Surgical Pathology (2nd ed.). Saunders. pp. 97. ISBN 978-0443066450.
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