Authors: J. Ďuraš 1; V. Procházka 2; A. Sýkorová 3 Authors‘ workplace: Klinika hematoonkologie LF OU a FN Ostrava 1; Hemato-onkologická klinika LF UP a FN Olomouc 2; IV. interní hematologická klinika LF UK a FN Hradec Králové 3 Published in: Transfuze Hematol. dnes,31, 2025, No. 4, p. 287-295. Category: Review/Educational Papers doi: https://doi.org/10.48095/cctahd202525
Marginal zone lymphomas (MZL) are indolent B-cell lymphomas arising from marginal zone B lymphocytes of germinal centre follicles in extranodal tissues, spleen, or lymph nodes. They represent the third most common group of non-Hodgkin lymphomas in the Czech Republic. In recent years, significant progress has been made in the understanding and management of relapsed or refractory (R/R) disease. Beyond immunochemotherapy, the combination of lenalidomide with rituximab and Bruton’s tyrosine kinase inhibitors (BTKi), particularly zanubrutinib, are already used in routine clinical practice. In the phase IIIb MAGNIFY study in 76 R/R MZL patients, lenalidomide plus rituximab achieved an overall response rate (ORR) of 64% and a complete remission (CR) rate of 39%, with a median progression-free survival (PFS) of 41.2 months. In the phase II MAGNOLIA study, zanubrutinib after ≥ 1 prior therapy achieved ORR 68.2%, CR 25.8%, and a 2-year PFS 70.9%, with a favourable safety profile. Data on other BTKi, acalabrutinib and pirtobrutinib, are limited to smaller phase II studies. Use of phosphatidylinositol 3-kinase inhibitors is hampered by the immune-mediated toxicity. CAR-T cell therapy, particularly liso-cel, appears highly promising. In the TRANSCEND FL study in patients with a median of 3 prior treatment lines, liso-cel achieved ORR 95.5%, CR 62.1%, and a 2-year PFS of 85.7%. Data on bispecific antibodies in MZL are limited. Most information is available for odronextamab, which in the phase II ELM-2 study after ≥ 2 prior systemic therapies achieved ORR and CR of 79.3%. The antibody–drug conjugate loncastuximab tesirine also shows encouraging results. Ongoing research is exploring combinations such as lenalidomide with bispecific antibodies, lenalidomide with tafasitamab, and BTKi with anti-CD20 antibodies. Among the therapies discussed, zanubrutinib is currently the only agent approved in Europe for R/R MZL. In the Czech Republic, none of these drugs has established reimbursement, and their use requires individual approval by the health insurance provider.
relapse – therapy – marginal zone lymphoma
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PODÍL AUTORŮ NA PŘÍPRAVĚ RUKOPISU
JD – příprava rukopisu (65 %)
AS – připomínkování a úpravy rukopisu (30 %)
VP – připomínkování finálního rukopisu (5 %)
PROHLÁŠENÍ O KONFLIKTU ZÁJMŮ
JD – autor v uplynulých 24 měsících poskytoval poradenskou a konzultační činnost pro firmy AstraZeneca, Bristol Myers Squibb, Eli Lilly, Johnson a Johnson, Roche, Swixx Biopharma,
VP – autor v uplynulých 24 měsících poskytoval poradenskou a konzultační činnost pro firmy Bristol Myers Squibb, Eli Lilly, Johnson a Johnson, Roche, Swixx Biopharm
AS – autorka v uplynulých 24 měsících poskytovala poradenskou a konzultační činnost pro firmy Gilead, Roche
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