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Leukemic Variant of Mantle Cell Lymphoma: Clinical Presentation and Management

  • Leukemia (A Aguayo, Section Editor)
  • Published:
Current Oncology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

This review summarizes the unique presentation and management of the leukemic variant of mantle cell lymphoma (LV-MCL, also referred to as non-nodal MCL) and highlights the biologic and clinical differentiation from classical mantle cell lymphoma (cMCL) in biomarker expression, clinical features, prognosis, disease course, and treatment.

Recent Findings

Several studies have evaluated the gene expression profile of mantle cell lymphoma, differentiating LV-MCL from cMCL. The typical immunophenotypic profile is CD5-positive, SOX 11-negative, CD23-low, CD200-low, and cyclin D1 overexpressed. LV-MCL commonly has mutated immunoglobulin heavy chain variable region genes. Data on treatment of LV-MCL is limited to retrospective analyses; the ideal treatment for these patients is unknown although many have a clinically indolent, asymptomatic presentation and often may be observed for an extended period without active treatment.

Summary

LV-MCL is a clinically and biologically distinct entity. Clinically, it must be distinguished from chronic lymphocytic leukemia and cMCL. Future prospective, randomized clinical trials are required to optimize management, define the initial treatment, and appropriately sequence treatment modalities.

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Authors and Affiliations

  1. Division of Hematology/Oncology, Department of Medicine, University of Virginia Cancer Center, Jefferson Park Avenue, PO 800716, Charlottesville, VA, 22908, USA

    Krista M. Isaac, Craig A. Portell & Michael E. Williams

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  1. Krista M. Isaac
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Correspondence to Michael E. Williams.

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Conflict of Interest

Krista M. Isaac declares that she has no conflict of interest. Craig A. Portell has received research funding from AbbVie, BeiGene, Kite/Gilead, Genentech, TG Therapeutics, Acerta, Infinity Pharmaceuticals/Verastem Oncology, and VelosBio and has received compensation from Amgen, BeiGene, Janssen, Pharmacyclics, Kite Pharma/Gilead Sciences, MorphoSys, and Genentech for service as a consultant. Michael E. Williams has received clinical trial support (paid to the University of Virginia) from Janssen, Pharmacyclics, TG Therapeutics, Gilead Sciences, and Celgene; has contributed to a lymphoma registry sponsored by Allos Therapeutics; has received speaker’s honoraria from Xian Janssen Pharmaceutical Ltd.; and has received compensation from Celgene, Gilead Sciences, TG Therapeutics, AbbVie, Kite Pharma, Juno Pharmaceuticals, Verastem Oncology, Seattle Genetics, Sandoz, AstraZeneca, and Kymera Therapeutics for participation on advisory boards.

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Isaac, K.M., Portell, C.A. & Williams, M.E. Leukemic Variant of Mantle Cell Lymphoma: Clinical Presentation and Management. Curr Oncol Rep 23, 102 (2021). https://doi.org/10.1007/s11912-021-01094-y

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