A uncommon type of B-cell non-Hodgkin lymphoma (NHL), Mantle Cell Lymphoma (MCL) is characterised by an aggressive clinical path and a poor prognosis that remains incurable for the majority of patients. The pathogenic hallmark of MCL disease is cyclin D1 overexpression, which results from t(11; 14)(q13; q32), and causes cell cycle disruption. In the WHO update, MCL was divided into two major subgroups, nodal and leukemic non-nodal MCL, based on lymphoid malignancies; each form has a specific clinical appearance and distinct molecular features. SOX11 is over-expressed in the nodal MCL subtype, while SOX11 negativity is correlated with the leukaemic non-nodal subtype. Including other forms of low-grade lymphoma, most commonly chronic lymphocytic leukaemia (CLL), follicular lymphoma (FL), marginal zone lymphoma (MZL), and lymphoblastic lymphoma, MCL has a broad variety of differential diagnoses (LBL). Therefore in this unusual subtype of NHL, correct histological biopsy diagnosis is paramount. With particular cytogenetic defects, MCL has a distinctive clinical appearance and unique morphological and immunophenotypic features. In general, this tumour has a poor prognosis and needs aggressive and novel treatment, so a correct diagnosis of MCL is of great importance. Conversely, for observation, the indolent leukemic variant MCL should be considered. There is a large differential diagnosis that can be misinterpreted as other forms of NHL for MCL and morphologic findings. The molecular basis of MCL highlights the biological role of novel therapies as diagnostic and prognostic aids and as targets. Recent advances in molecular and cytogenetic research have increased the precision of the diagnosis of MCL and improved the prognosis of the disease. B cell receptor inhibitors have in addition, revolutionised the treatment of MCL. An accurate diagnosis of MCL is therefore very important since aggressive and novel targeted therapy may be needed for this.
Author(s) Details
Haematology Department, University Hospital Waterford, South East Cancer Center, Waterford, Ireland.
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