Difference between revisions of "Small cell lymphomas"

From Libre Pathology
Jump to navigation Jump to search
(split out)
(hive off hairy cell leukemia to its own page)
 
(12 intermediate revisions by 2 users not shown)
Line 20: Line 20:
! Other
! Other
|-
|-
| Follicular lymphoma
| [[Follicular lymphoma]]
| Follicle
| Follicle
| Small, centrocytes, centroblasts
| Small, centrocytes, centroblasts
Line 33: Line 33:
| 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]]
|-
|-
Line 70: Line 70:
|}
|}


Medium and large cell lymphomas:
Common medium and large cell lymphomas:
{| class="wikitable"
{| class="wikitable"
! Name
! Name
Line 112: Line 112:
==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====
Classification:
<gallery>
#Extranodal marginal zone lymphoma.
Image:Gastrointestinal_lymphoepithelial_lesion_-_very_high_mag.jpg | Lymphoepithelial lesion - very high mag. (WC)
#*If in ''mucosa-associated lymphoid tissue'' known as a ''MALT lymphoma'', [[AKA]] ''MALToma''.
Image:Gastrointestinal_lymphoepithelial_lesion_-_intermed_mag.jpg | Lymphoepithelial lesion - intermed. mag. (WC)
#Splenic marginal zone lymphoma (SMZL).
</gallery>
#Nodal marginal zone lymphoma (NMZL).
www:
*[http://path.upmc.edu/cases/case405.html MALT lymphoma - several images (upmc.edu)].
*[http://www.pathpedia.com/education/eatlas/histopathology/intestine_large/lymphoma_malt-type.aspx MALT lymphoma - several images (pathpedia.com)].
 
===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.


==Hairy cell leukemia==
==Hairy cell leukemia==
*Abbreviated ''HCL''.
See [[Hairy cell leukemia]]
===General===
*Name comes from appearance on blood smear - cell hairy.
*Do to the biology, ''dry taps'' are common.<ref name=pmid22499303>{{Cite journal  | last1 = Galani | first1 = KS. | last2 = Subramanian | first2 = PG. | last3 = Gadage | first3 = VS. | last4 = Rahman | first4 = K. | last5 = Ashok Kumar | first5 = MS. | last6 = Shinde | first6 = S. | last7 = Mahadik | first7 = S. | last8 = Ansari | first8 = R. | last9 = Sengar | first9 = M. | title = Clinico-pathological profile of Hairy cell leukemia: critical insights gained at a tertiary care cancer hospital. | journal = Indian J Pathol Microbiol | volume = 55 | issue = 1 | pages = 61-5 | month =  | year =  | doi = 10.4103/0377-4929.94858 | PMID = 22499303 }}</ref>
 
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====
<gallery>
Image:Hairy_cell_leukemia.jpg | HCL - blood film. (WC)
Image:Hairy cell leukemia - high mag.jpg | HCL - high mag. (WC)
Image:Hairy_cell_leukemia_-_very_high_mag.jpg | HCL - very high mag. (WC)
</gallery>
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====
<gallery>
Image:Chronic lymphocytic leukemia - low mag.jpg | CLL - low mag. (WC)
Image:Chronic lymphocytic leukemia - intermed mag.jpg | CLL - intermed. mag. (WC)
Image:Chronic lymphocytic leukemia - high mag.jpg | CLL - high mag. (WC)
Image:Chronic lymphocytic leukemia - very high mag.jpg | CLL - very high mag. (WC)
</gallery>
www:
*[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 ==
Line 339: Line 175:


[[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.

Mantle cell lymphoma

  • Abbreviated MCL.

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

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.