How I treat double-hit lymphoma

Jonathan W. Friedberg

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  • Therapy for double-hit transformation of follicular lymphoma
    • Gareth P Gregory, Haematologist Monash Health and School of Clinical Sciences at Monash University, Clayton, Victoria, Australia
    • Other Contributors:
      • Aditya Tedjaseputra, Hospital Medical Officer
      • Stephen S Opat, Director and Professor, Clinical Haematology

    We read with interest the article How I treat double-hit lymphoma by Professor Friedberg[1] and are grateful for this timely contribution. We seek to clarify the setting of transformed follicular lymphoma (FL) in which a MYC rearrangement is acquired. The 2016 revision of the World Health Organisation classification of lymphoid neoplasms specifically defines the new entity of high-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 translocations as the new category for double-/triple-hit lymphomas exclusive of FL or plasmablastic lymphoma[2]. We feel that this distinction is important as the biology and response to therapy of de novo double-hit DLBCL differs from that of an acquired MYC translocation in the setting of transformation of FL.

    As the majority of this patient group tend to be older and frail, we have favored R-CHOP for treatment-naïve FL patients at transformation (with or without maintenance rituximab) and observed favorable outcomes with this schedule. This approach is supported by retrospective studies of treatment-naïve patients treated with R-CHOP at transformation demonstrating outcomes comparable to de novo DLBCL, in stark contrast to the very poor prognosis conferred by transformation in the setting of previously-treated FL[3,4]. While these cohorts were not restricted to patients with MYC translocation at transformation, elegant genomic studies have demonstrated up to 25% of transformed follicular lymphomas harbor translocations of MYC, and up...

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    Conflict of Interest:
    None declared.