Though multiple relapses and serial shortening of remission is one of the characteristics of follicular lymphoma (FL), standard third- and later-line treatments with clear evidence have not yet been established. Tazemetostat, the first oral enhancer of zester homolog 2 (EZH2) inhibitor, showed a favorable clinical outcome and safety profile against relapsed mutant EZH2 FL in a clinical trial and was applied to this clinical setting. Peripheral blood involvement, known as the leukemic phase, was observed in approximately 10% of patients with FL and reported as a poor prognostic factor. However, because of the infrequency of EZH2-activating mutations, clinical data on tazemetostat against FL in the leukemic phase is lacking. Herein, we report a case of multiple relapsed FL in the leukemic phase for which tazemetostat was administered as a sixth-line treatment. Tazemetostat monotherapy showed a slow and sustained clinical efficacy in the leukemic phase as shown by nodal involvement. Circulating lymphoma cells gradually decreased and disappeared in counts after 4 months of treatment. However, circulating lymphoma cells were still detected by flow cytometry up to 6 months of treatment and finally undetected after 9 months. Extended-interval dosing of tazemetostat transformed a partial response into a complete response. Thus, tazemetostat is effective for the treatment of multiple relapsed FL in the leukemic phase.
Keywords: EZH2 inhibitor; Follicular lymphoma; Multiple relapses; Tazemetostat.
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