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Marginal zone lymphoma (view source)
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# | '''Marginal zone lymphoma''', abbreviated '''MZL''', is a common type of lymphoma composed of [[small cell lymphomas|small cells]]. | ||
==General== | |||
*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> | |||
DDx: | |||
*Other [[small cell lymphomas]]. | |||
*[[DLBCL]] - should be a distinctive region at low power.{{fact}} | |||
===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. | |||
==See also== | |||
*[[Small cell lymphomas]]. | |||
*[[Lymphoma]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Small cell lymphomas]] | |||