Mantle cell lymphoma classification

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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:

1. Classical MCL.
2. Leukemic nonnodal MCL.
3. 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:
  1. Blastoid MCL
  2. 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.