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. 2021 Jul;194(2):382-392.
doi: 10.1111/bjh.17550. Epub 2021 Jun 21.

Adverse outcome in follicular lymphoma is associated with MYC rearrangements but not MYC extra copies

Lucile Bussot  1 , Simon Chevalier  2   3   4 , Justine Cristante  3 , Béatrice Grange  5 , Bruno Tesson  6 , Clémence Deteix-Santana  7 , Frédérique Orsini-Piocelle  8 , Cécile Leyronnas  9 , Sophie Dupire  10 , Rémy Gressin  1   4 , Gilles Salles  11   12   13 , Emmanuel Bachy  11   12   13 , Anouk Emadali  4 , Séverine Valmary-Degano  3   14 , Sarah Huet  12   13   15 , Christine Lefebvre  4   16 , Sylvain Carras  1   3   4   16
Affiliations

Adverse outcome in follicular lymphoma is associated with MYC rearrangements but not MYC extra copies

Lucile Bussot et al. Br J Haematol. 2021 Jul.

Abstract

Follicular lymphomas (FLs) with MYC rearrangements (MYC-R) and extra copies of MYC (MYC-EC) are rare and the prognosis impact is uncertain. We conducted a retrospective study including 321 FL patients, among whom 259 (81%) had no 8q24 alterations and 62 (19%) were assigned to 8qAlt. Forty-five cases were classified as MYC-EC and six as MYC-R. MYC-R patients were significantly older (P = 0·008), had higher follicular lymphoma international prognostic index (FLIPI) stage (P = 0·05) and β2-microglobulin (β2m; P = 0·05). Among patients treated with immuno-chemotherapy, four presented a MYC-R and 25 a MYC-EC. Univariate analysis showed the absence of significant difference between MYC-EC and normal MYC (MYC-NL) regarding progression-free survival (PFS; HR1·3; 95% CI [0·4-1·6]) and specific overall survival (SOS; HR 1·6; 95% CI [0·4-5·7]). Those results were compared to data from the PRIMA trial. This confirmed that MYC-EC had no impact on PFS (P = 0·86) or SOS (P = 0·9). Conversely, MYC-R was associated with a trend to inferior outcome regarding PFS (HR : 6·1; 95% CI [2·2-17·1]; P = 0·00026), lymphoma-related death (SOS; HR 13·6; 95% CI [2·9-65]; P = 0·00014) and risk of transformation (transformation-free survival (TFS); HR 82·7; 95% CI [14·8-463·4]; P < 0·0001). In conclusion, MYC-EC has no prognostic impact in FL but MYC-R FL tended to be associated with an increased risk of transformation and poorer outcome.

Keywords: MYC; follicular lymphoma; prognosis; rearrangement; transformation.

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