Preface: why this special series on marginal zone lymphomas?
Marginal zone lymphoma (MZL) is a relatively uncommon form of indolent non-Hodgkin lymphoma currently defined anatomically as extranodal, nodal and splenic. The therapeutic approaches are variable, ranging from antimicrobial approaches to monoclonal antibodies, chemoimmunotherapy, kinase inhibitors and cellular therapies. From April 17–18, 2019, the Lymphoma Research Foundation (LRF) gathered international experts including members of the International Extranodal Study Group in the field of MZL in New York City for the inaugural International Scientific Workshop on Marginal Zone Lymphoma. This group collectively realized that there was far more to be learned and accomplished in MZL.
One impediment to therapeutic progress in MZL is that, MZL and follicular lymphoma (FL) patients are often included in the same protocols despite their differences in biology and natural history. Thus, a number of questions were addressed: within MZL is the biology of extranodal, nodal and splenic subtypes different? Can broader epidemiology studies contribute to a better understanding of these diseases? How can further understanding of the biology of transformation aid in the management strategies of MZL? Would it be preferable for MZL and FL patients be included in the different clinical trials? In extranodal MZL, should refinements in measurable disease criteria such as orbital disease be developed in certain subsets? What is the optimal radiation therapy approach in the different subsets of MZL? What future directions can enhance design and accrual to clinical trials?
A number of conclusions were drawn from this Workshop. Given the differences in biology and natural history of MZL and FL, clinical protocols should not lump these entities together as low-grade lymphoma. Because the rarity of the diseases, collaborative efforts are imperative. Further delineation of the cell of origin of MZL cells is critical to further development of therapeutic strategies to improve outcomes in the various subsets of MZL. Since MZL is currently considered incurable, patients are subjected to a series of treatment options. A better understanding of the molecular etiology will help individualize therapy and direct patients to the optimal sequence of the novel targeted treatment options becoming available.
One outcome of this Workshop is this focused issue on MZL which serves as the most extensive and comprehensive review of individual topics in MZL by international leaders in the field. We hope that it will provide a roadmap taking us from where the field currently is, to where it is going, but, more importantly where it will need to go to lead to the cure of patients with MZL.
Acknowledgments
Funding: None.
Footnote
Provenance and Peer Review: This article was commissioned by the editorial office, Annals of Lymphoma for the series “Marginal Zone Lymphomas”. The article did not undergo external peer review.
Conflicts of Interest: The authors have completed the ICMJE uniform disclosure form (available at https://dx.doi.org/10.21037/aol-21-22). The series “Marginal Zone Lymphomas” was commissioned by the editorial office without any funding sponsorship. TMH, DR, FB and EZ served as the unpaid Guest Editors of the series. EZ serves as an unpaid editorial board member of Annals of Lymphoma. TMH reports his participation on Advisory Board for Sea Gen, Tess therapeutics, Loxo Oncology at Lilly, Morphosys, Incyte. Dr. DR reports grants and personal fees received from AstraZeneca, Janssen, Abbvie, outside the submitted work. Dr. FB reports institutional research funds received from Acerta, ADC Therapeutics, Bayer AG, Cellestia, CTI Life Sciences, EMD Serono, Helsinn, ImmunoGen, Menarini Ricerche, NEOMED Therapeutics 1, Nordic Nanovector ASA, Oncology Therapeutic Development, PIQUR Therapeutics AG, consulting fee from Helsinn, Menarini Ricerche, payments for expert statements from HTG, and travel grant from PIQUR Therapeutics AG, Amgen, Astra Zeneca, Jazz Pharmaceuticals. Dr. EZ reports grants to institution from AstraZeneca, Celgene, Incyte, Roche and consulting fees received from Beigene, Celgene, Incyte, Merck, Roche, Celltrion Healthcare, Kite (Gilead Company). The authors have no other conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
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Cite this article as: Habermann TM, Rossi D, Bertoni F, Coleman M, Zucca E. Preface: why this special series on marginal zone lymphomas? Ann Lymphoma 2021;5:21.
