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Difference between revisions of "Mantle cell lymphoma"
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| Synonyms = | | Synonyms = | ||
| Micro = small monomorphic (uniform size, shape and staining) lymphoid population with abundant mitoses, +/-scattered epithelioid histiocytes (should not be confused with tingible-body macrophages), +/-sclerosed blood vessels | | Micro = small monomorphic (uniform size, shape and staining) lymphoid population with abundant mitoses, +/-scattered epithelioid histiocytes (should not be confused with tingible-body macrophages), +/-sclerosed blood vessels | ||
| Subtypes = | | Subtypes = blastic variant | ||
| LMDDx = other [[small cell lymphomas]] (esp. [[MALT lymphoma]]), [[Burkitt lymphoma]] | | LMDDx = other [[small cell lymphomas]] (esp. [[MALT lymphoma]]), [[Burkitt lymphoma]] | ||
| Stains = | | Stains = | ||
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| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = poor | | Prognosis = moderately aggressive to poor | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = | ||
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==General== | ==General== | ||
*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> | *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> | ||
*Rare ~ 2% of non-Hogkin's lymphoma in a series of over 4000 patients.<ref name=pmid18723950>{{cite journal |author=Gujral S, Agarwal A, Gota V, ''et al.'' |title=A clinicopathologic study of mantle cell lymphoma in a single center study in India |journal=Indian J Pathol Microbiol |volume=51 |issue=3 |pages=315–22 |year=2008 |pmid=18723950 |doi= |url=}}</ref> | |||
GI tract - typically:<ref name=pmid22236942/> | GI tract - typically:<ref name=pmid22236942/> | ||
*Abdominal pain (~37% of cases) or GI bleeding (~26% of cases). | *Abdominal pain (~37% of cases) or GI bleeding (~26% of cases). | ||
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==Microscopic== | ==Microscopic== | ||
Features:<ref>Good, D. 17 August 2010.</ref> | Features:<ref>Good, D. 17 August 2010.</ref> | ||
* | *Typically a small monomorphic (uniform size, shape and staining) lymphoid population. | ||
**May be "large" (blastic variant of mantle cell lymphoma) with 1 or 2 nucleoli.<ref name=pmid17917091>{{cite journal |author=Todorovic M, Pavlovic M, Balint B, ''et al.'' |title=Immunophenotypic profile and clinical characteristics in patients with advanced stage mantle cell lymphoma |journal=Med. Oncol. |volume=24 |issue=4 |pages=413–8 |year=2007 |pmid=17917091 |doi= |url=}}</ref> | |||
*Abundant mitoses. | *Abundant mitoses. | ||
*Scattered epithelioid histiocytes (should not be confused with tingible-body macrophages). | *Scattered epithelioid histiocytes (should not be confused with tingible-body macrophages). | ||
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*Other [[small cell lymphomas]], esp. [[marginal zone lymphoma]]. | *Other [[small cell lymphomas]], esp. [[marginal zone lymphoma]]. | ||
*[[Burkitt's lymphoma]]. | *[[Burkitt's lymphoma]]. | ||
*Large B-cell lymphoma - for blastic variant of MCL.<ref name=pmid11681422>{{cite journal |author=Bernard M, Gressin R, Lefrère F, ''et al.'' |title=Blastic variant of mantle cell lymphoma: a rare but highly aggressive subtype |journal=Leukemia |volume=15 |issue=11 |pages=1785–91 |year=2001 |month=November |pmid=11681422 |doi= |url=}}</ref> | |||
===Images=== | ===Images=== | ||
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www: | www: | ||
*[http://path.upmc.edu/cases/case441.html MCL - several images (upmc.edu)]. | *[http://path.upmc.edu/cases/case441.html MCL - several images (upmc.edu)]. | ||
[[File:4 718053471 sl 1.png|Mantle cell lymphoma]] | |||
[[File:4 718053471 sl 2.png|Mantle cell lymphoma]] | |||
[[File:4 718053471 sl 3.png|Mantle cell lymphoma]] | |||
[[File:4 718053471 sl 4.png|Mantle cell lymphoma]] | |||
[[File:4 718053471 sl 5.png|Mantle cell lymphoma]] | |||
[[File:4 718053471 sl 6.png|Mantle cell lymphoma]] | |||
[[File:4 718053471 sl 7.png|Mantle cell lymphoma]]<br> | |||
Mantle cell lymphoma in a 56 year old woman. A. A blue sea of cells spares a lymphoid follicle in the upper right corner. B. Small, round to reniform cells contrast with the larger tingible body macrophage (arrow). C-F. Tumor cells are Cyclin D1. CD20, BCL2, and CD5 positive positive. G. About 60% of tumor cells were positive for Ki67. Tumor cells were negative for CD3, CD10, and BCL6. | |||
==IHC== | ==IHC== | ||
*CD5 +ve. | *CD45 +ve. | ||
*CD20 +ve. | |||
*CD79a +ve. | |||
*CD5 +ve -- '''important'''. | |||
**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> | **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. | *CD43 +ve. | ||
*Cyclin D1 +ve. | *Cyclin D1 +ve -- '''key immunostain'''. | ||
**[[AKA]] BCL1, AKA CCND1.<ref name=omim168461>{{OMIM|168461}}</ref> | **[[AKA]] BCL1, AKA CCND1.<ref name=omim168461>{{OMIM|168461}}</ref> | ||
Others: | Others: | ||
*CD23 -ve. | *CD23 -ve. | ||
**Positive in CLL. | **Positive in [[CLL]]. | ||
==Molecular== | ==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> | *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> | ||
==Sign out== | |||
<pre> | |||
TERMINAL ILEUM, BIOPSY: | |||
- MANTLE CELL LYMPHOMA. | |||
COMMENT: | |||
The tumour consists of small lymphoid cells in sheets. | |||
The tumour stains as follows: | |||
POSITIVE: CD20, CD5, BCL2, cyclin D1. | |||
NEGATIVE: CD23, BCL6, CD10. | |||
PROLIFERATION (Ki-67): 25%. | |||
</pre> | |||
==See also== | ==See also== | ||
*[[Small cell lymphomas]]. | *[[Small cell lymphomas]]. | ||
*[[Ileal nodular lymphoid hyperplasia]]. | |||
==References== | ==References== | ||