Breakthrough Treatment for Mantle Cell Lymphoma
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2017, Vol 1, Issue 4
Abstract
MCL has a dismal prognosis (“the worst lymphoma to have”), with a median OS rate of 3 years only. Prognosis of limited stage disease is almost similar to that of stage lll MCL No curative therapy has been established so far. The US FDA granted breakthrough therapy designations to a bruton tyrosine kinase inhibitor, acalabrutinib in MCL patients who have previously received at least one line of therapy. Furthermore, the combination of bortezomib and a retinoid compound, fenretinide is synergistically cytotoxic against MCL lines and warrants further evaluation in vivo and in clinical trials. In addition, the combination of anti-Mcl-1 lipidoid nanoparticles with other forms of targeted therapy offers hope for reducing or replacing cytotoxic chemotherapy as standard treatment for MCL that over express Mcl-1MCL is an aggressive small B-cell lymphoma [1] that is derived from naïve, pre-germinal center cells of primary follicles or mantle regions of secondary follicles [2]. It constitutes nearly 6-8% of all NHL in Europe and North America. The median age at diagnosis is 65 years with a male preponderance of 3 to 1 [3]. These patients are typically Caucasian (about 2:1) [4]. A family history of hematopoietic malignancies has been linked with a 2-fold increased risk of MCL. The risk of MCL is linked with European strains of the Borrelia burgdorferi infection particularly when manifesting as acrodermatitis atrophicans. But, there is still a lack of solid evidence for such association. Body mass index, cigarette smoking, alcohol intake and severe immune suppression have not been implicated as risk factors for MCL [4].MCL subtypesTwo subtypes with different clinicopathological manifestations and molecular pathogenetic pathways are recognized: Classical one largely with unmutated/minimally mutated IGHV and mostly SOX11+ and typically involves lymph nodes and other extranodal sites. The other is an indolent form, largely with mutated IGHV and mostly SOX11− [1]. It is characterized by non-nodal leukemic presentation with mild to moderate lymphocytosis. Furthermore, these cases are associated with low Ki67 (≤10%) and kappa light chain expression [2]
Authors and Affiliations
Nahla A M Hamed
Keywords
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- EP ID EP566311
- DOI 10.26717/BJSTR.2017.01.000322
- Views 298
- Downloads 0
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