Mantle cell lymphoma classification: Difference between revisions
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=== Classical MCL: === | === Classical MCL: === | ||
* Classical MCL typically | * Classical MCL typically develops from immunoglobulin heavy-chain variable region gene(IGHV)-unmutated or minimally mutated B cells that usually express SOX11 (SRY-Box 11 gene). | ||
* Classical MCL usually involves the lymph nodes and other extranodal sites. | * Classical MCL usually involves the lymph nodes and other extranodal sites. | ||
* However, these cells can acquire further molecular and cytogenetic abnormalities leading to the more aggressive: | * However, these cells can acquire further molecular and cytogenetic abnormalities leading to the more aggressive: | ||
*#Blastoid MCL | *#Blastoid MCL | ||
*#Pleomorphic MCL | *#Pleomorphic MCL | ||
=== Leukemic nonnodal MCL: === | |||
* Leukemic nonnodal MCL typically develops from immunoglobulin heavy-chain variable region gene(IGHV)-mutated B cells that do not express SOX11 (SRY-Box 11 gene). | |||
* Leukemic nonnodal MCL involve the peripheral blood (PB), bone marrow and the spleen. | |||
* Although these are commonly clinically indolent in nature further mutations ,especially of TP53, can lead to more aggressive disease. | |||
=== In-situ mantle cell neoplasia (ISMCN): === | |||
* It is characterized by the presence of cyclin D1+ cells mostly in the inner mantle zones of follicles in lymphoid tissues that do not otherwise suggest the diagnosis of a MCL. | |||
* The in situ lesion stage may be a common step in both SOX11-negative and -positive subtypes of MCL as some in situ lesions express SOX11, whereas others are SOX11 negative. | |||
* ISMCN is often found incidentally, occasionally in association with other types of lymphomas. | |||
==References== | ==References== | ||
Revision as of 18:10, 6 November 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ali Akram, M.B.B.S.[2]
Overview
According to the revised 2016 World Health Organization classification of lymphoid neoplasms, mantle cell lymphoma(MCL) can be broadly classified into three types:
- Classical MCL.
- Leukemic nonnodal MCL.
- In-situ mantle cell neoplasia (ISMCN).
Classification
Classical MCL:
- Classical MCL typically develops from immunoglobulin heavy-chain variable region gene(IGHV)-unmutated or minimally mutated B cells that usually express SOX11 (SRY-Box 11 gene).
- Classical MCL usually involves the lymph nodes and other extranodal sites.
- However, these cells can acquire further molecular and cytogenetic abnormalities leading to the more aggressive:
- Blastoid MCL
- Pleomorphic MCL
Leukemic nonnodal MCL:
- Leukemic nonnodal MCL typically develops from immunoglobulin heavy-chain variable region gene(IGHV)-mutated B cells that do not express SOX11 (SRY-Box 11 gene).
- Leukemic nonnodal MCL involve the peripheral blood (PB), bone marrow and the spleen.
- Although these are commonly clinically indolent in nature further mutations ,especially of TP53, can lead to more aggressive disease.
In-situ mantle cell neoplasia (ISMCN):
- It is characterized by the presence of cyclin D1+ cells mostly in the inner mantle zones of follicles in lymphoid tissues that do not otherwise suggest the diagnosis of a MCL.
- The in situ lesion stage may be a common step in both SOX11-negative and -positive subtypes of MCL as some in situ lesions express SOX11, whereas others are SOX11 negative.
- ISMCN is often found incidentally, occasionally in association with other types of lymphomas.