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

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


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Gene Duplication
  • Gene Rearrangement*
  • Humans
  • Lymphoma, Follicular / diagnosis
  • Lymphoma, Follicular / genetics*
  • Lymphoma, Follicular / therapy
  • Middle Aged
  • Prognosis
  • Progression-Free Survival
  • Proto-Oncogene Proteins c-myc / genetics*
  • Retrospective Studies
  • Young Adult


  • MYC protein, human
  • Proto-Oncogene Proteins c-myc