Difference between revisions of "Small cell lymphomas"

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Medium and large cell lymphomas:
Common medium and large cell lymphomas:
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==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===
Classification:
*Comes in three different flavours:
#Extranodal marginal zone lymphoma.
*#Extranodal marginal zone lymphoma.
#*If in ''mucosa-associated lymphoid tissue'' known as a ''MALT lymphoma'', [[AKA]] ''MALToma''.
*#*If in ''mucosa-associated lymphoid tissue'' known as a ''MALT lymphoma'', [[AKA]] ''MALToma''.
#Splenic marginal zone lymphoma (SMZL).
*#Splenic marginal zone lymphoma (SMZL).
#Nodal marginal zone lymphoma (NMZL).
*#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====
<gallery>
Image:Gastrointestinal_lymphoepithelial_lesion_-_very_high_mag.jpg | Lymphoepithelial lesion - very high mag. (WC)
Image:Gastrointestinal_lymphoepithelial_lesion_-_intermed_mag.jpg | Lymphoepithelial lesion - intermed. mag. (WC)
</gallery>
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>{{Cite journal  | last1 = Vitolo | first1 = U. | last2 = Ferreri | first2 = AJ. | last3 = Montoto | first3 = S. | title = Follicular lymphomas. | journal = Crit Rev Oncol Hematol | volume = 66 | issue = 3 | pages = 248-61 | month = Jun | year = 2008 | doi = 10.1016/j.critrevonc.2008.01.014 | PMID = 18359244 }}</ref>
*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 ==

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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