Difference between revisions of "Mantle cell lymphoma"

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#redirect [[small cell lymphomas]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Mantle cell lymphoma - intermed mag.jpg
| Width      =
| Caption    = Mantle cell lymphoma. [[H&E stain]].
| 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
| Subtypes  = blastic variant
| LMDDx      = other [[small cell lymphomas]] (esp. [[MALT lymphoma]]), [[Burkitt lymphoma]]
| Stains    =
| IHC        = cyclin D1 +ve, CD5 +ve, CD43 +ve, CD20 +ve, CD23 -ve
| EM        =
| Molecular  = t(11;14)(q13;q32) / IGH-CCND1
| IF        =
| Gross      =
| Grossing  =
| Site      = [[lymph node]], GI tract, other sites
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = not common
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = moderately aggressive to poor
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Mantle cell lymphoma''', abbreviated '''MCL''', is less common [[small cell lymphoma]] that is relatively aggressive when compared to other small cell lymphomas.
 
==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>
*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/>
*Abdominal pain (~37% of cases) or GI bleeding (~26% of cases).
 
==Gross==
*GI tract: polypoid lesions (~50% of cases).<ref name=pmid22236942>{{Cite journal  | last1 = Kim | first1 = JH. | last2 = Jung | first2 = HW. | last3 = Kang | first3 = KJ. | last4 = Min | first4 = BH. | last5 = Lee | first5 = JH. | last6 = Chang | first6 = DK. | last7 = Kim | first7 = YH. | last8 = Son | first8 = HJ. | last9 = Rhee | first9 = PL. | title = Endoscopic findings in mantle cell lymphoma with gastrointestinal tract involvement. | journal = Acta Haematol | volume = 127 | issue = 3 | pages = 129-34 | month =  | year = 2012 | doi = 10.1159/000333139 | PMID = 22236942 }}</ref>
 
==Microscopic==
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.
*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]].
*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===
<gallery>
Image:Mantle cell lymphoma - low mag.jpg | MCL - low mag. (WC)
Image:Mantle cell lymphoma - intermed mag.jpg | MCL - intermed. mag. (WC)
Image:Mantle cell lymphoma - low mag - cyclin D1.jpg | MCL - low mag. - cyclin D1. (WC)
Image:Mantle cell lymphoma - intermed mag - cyclin D1.jpg | MCL - intermed. mag. - cyclin D1. (WC)
</gallery>
www:
*[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==
*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>
*CD43 +ve.
*Cyclin D1 +ve -- '''key immunostain'''.
**[[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>
 
==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==
*[[Small cell lymphomas]].
*[[Ileal nodular lymphoid hyperplasia]].
 
==References==
{{Reflist|2}}
 
[[Category:Diagnosis]]
[[Category:Small cell lymphomas]]

Latest revision as of 20:28, 24 February 2017

Mantle cell lymphoma
Diagnosis in short

Mantle cell lymphoma. H&E stain.
LM 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 blastic variant
LM DDx other small cell lymphomas (esp. MALT lymphoma), Burkitt lymphoma
IHC cyclin D1 +ve, CD5 +ve, CD43 +ve, CD20 +ve, CD23 -ve
Molecular t(11;14)(q13;q32) / IGH-CCND1
Site lymph node, GI tract, other sites
Prevalence not common
Prognosis moderately aggressive to poor

Mantle cell lymphoma, abbreviated MCL, is less common small cell lymphoma that is relatively aggressive when compared to other small cell lymphomas.

General

  • Relatively aggressive - guarded prognosis.[1]
  • Rare ~ 2% of non-Hogkin's lymphoma in a series of over 4000 patients.[2]

GI tract - typically:[3]

  • Abdominal pain (~37% of cases) or GI bleeding (~26% of cases).

Gross

  • GI tract: polypoid lesions (~50% of cases).[3]

Microscopic

Features:[4]

  • 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.[5]
  • Abundant mitoses.
  • Scattered epithelioid histiocytes (should not be confused with tingible-body macrophages).
  • Sclerosed blood vessels.

DDx:

Images

  • MCL - low mag. (WC)

  • MCL - intermed. mag. (WC)

  • MCL - low mag. - cyclin D1. (WC)

  • MCL - intermed. mag. - cyclin D1. (WC)

www:

Mantle cell lymphoma Mantle cell lymphoma Mantle cell lymphoma Mantle cell lymphoma Mantle cell lymphoma Mantle cell lymphoma Mantle cell lymphoma
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

  • CD45 +ve.
  • CD20 +ve.
  • CD79a +ve.
  • CD5 +ve -- important.
    • Negative in case reports.[7]
  • CD43 +ve.
  • Cyclin D1 +ve -- key immunostain.

Others:

  • CD23 -ve.
    • Positive in CLL.

Molecular

  • t(11;14)(q13;q32) / IGH-CCND1.[9]

Sign out

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%.

See also

References

  1. ↑ 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.
  2. ↑ Gujral S, Agarwal A, Gota V, et al. (2008). "A clinicopathologic study of mantle cell lymphoma in a single center study in India". Indian J Pathol Microbiol 51 (3): 315–22. PMID 18723950.
  3. ↑ 3.0 3.1 Kim, JH.; Jung, HW.; Kang, KJ.; Min, BH.; Lee, JH.; Chang, DK.; Kim, YH.; Son, HJ. et al. (2012). "Endoscopic findings in mantle cell lymphoma with gastrointestinal tract involvement.". Acta Haematol 127 (3): 129-34. doi:10.1159/000333139. PMID 22236942.
  4. ↑ Good, D. 17 August 2010.
  5. ↑ Todorovic M, Pavlovic M, Balint B, et al. (2007). "Immunophenotypic profile and clinical characteristics in patients with advanced stage mantle cell lymphoma". Med. Oncol. 24 (4): 413–8. PMID 17917091.
  6. ↑ Bernard M, Gressin R, Lefrère F, et al. (November 2001). "Blastic variant of mantle cell lymphoma: a rare but highly aggressive subtype". Leukemia 15 (11): 1785–91. PMID 11681422.
  7. ↑ URL: http://path.upmc.edu/cases/case308/dx.html. Accessed on: 14 January 2012.
  8. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 168461
  9. ↑ URL: http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html. Accessed on: 10 August 2010.
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