HaematologyTier 2Disease (DEADMAN)

Mantle cell lymphoma

Description

Mantle cell lymphoma - a B cell non-Hodgkin lymphoma characterised by the t(11;14) translocation causing cyclin D1 overexpression, typically presenting with widespread disease at diagnosis and historically considered to have a relatively aggressive course despite some overlapping features with indolent lymphomas

Predominantly affects older men; usually presents at an advanced stage (III/IV) with widespread lymphadenopathy, bone marrow, and often GI tract involvement (lymphomatous polyposis) at diagnosis

The t(11;14)(q13;q32) translocation juxtaposes the cyclin D1 gene with the immunoglobulin heavy chain enhancer, causing constitutive cyclin D1 overexpression - driving dysregulated cell cycle progression (cyclin D1 normally promotes G1-to-S phase transition) - cyclin D1 overexpression on immunohistochemistry is essentially diagnostic when combined with the characteristic morphology and immunophenotype (CD5+, CD23-negative - the CD23 negativity distinguishing it from CLL/SLL, which is also CD5+)

Lymph node biopsy with immunohistochemistry (cyclin D1, CD5, CD23) and/or FISH for t(11;14) confirms diagnosis
Staging (as for other lymphomas) includes CT/PET imaging and bone marrow biopsy; GI tract involvement is common and endoscopic assessment may be part of staging given the characteristic lymphomatous polyposis pattern

Treatment intensity is stratified by patient fitness: intensive chemoimmunotherapy +/- autologous stem cell transplant in fit patients (aiming for durable remission), less intensive chemoimmunotherapy in less fit/older patients
BTK inhibitors (ibrutinib and newer agents) have transformed relapsed/refractory mantle cell lymphoma management, targeting B cell receptor signalling
Despite generally responding well to initial treatment, mantle cell lymphoma has historically had a pattern of eventual relapse in most patients, distinguishing its natural history from both fully indolent lymphomas (which may never need treatment) and fully curable aggressive lymphomas

t(11;14) translocation and cyclin D1, CLL/SLL (the key CD5+ differential, distinguished by CD23 status), gastrointestinal lymphomatous polyposis

Mantle cell lymphoma occupies a distinctive niche between indolent and aggressive lymphoma behaviour - histologically it can appear deceptively low-grade, but its clinical course has traditionally been more aggressive than typical indolent lymphomas, a distinction increasingly nuanced by novel targeted agents improving outcomes

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