Prognosis of Follicular Lymphoma (FL)

FL typically has an indolent presentation, with a long estimated survival.1,2 Grade 3b FL seems to have a notably different prognosis or clinical course.3

  • FL is a chronic incurable condition, often with multiple relapses4
  • Median progression-free survival (PFS) for patients with newly diagnosed FL is approximately 7 years5*
  • Median overall survival (OS) can be 10-20 years1,6
  • Survival for patients with FL has improved over time6

Follicular Lymphoma International Prognostic Index (FLIPI)

NHL subtype, disease stage, and disease grade are all associated with each patient’s prognosis. Indices have been created that combine the relative input of disease stage and other prognostic factors—including age, ECOG performance status, and serum LDH—into a single score.8,9 Scores from these clinical criteria help physicians to determine prognosis.8,9

Prognostic indices vary between lymphoma subtypes. The prognostic index developed for FL is called the Follicular Lymphoma International Prognostic Index (FLIPI)10,11:

  • FLIPI-1 divides patients into 3 prognostic groups based on age, Ann Arbor stage, number of nodal sites involved, hemoglobin levels, and serum LDH levels10
  • FLIPI-2 is an updated version created in the era of chemoimmunotherapy, and includes age, hemoglobin levels, the longest diameter of largest involved lymph node, beta-2 microglobulin levels, and bone marrow involvement11

The FLIPI-1 and FLIPI-2 assign points for each prognostic factor present, and the cumulative scores are used to assess prognostic risk. Both indices have established 3 prognostic groups based on the cumulative score, with significantly different survival estimates.10,11

5-year survival rate (%) by risk score10,11


FLIPI: According to a study of 1795 patients initially diagnosed with follicular lymphoma between January 1, 1985 and December 31, 1992 at one of 27 European and American institutions.
FLIPI-2: According to a study of 1093 newly-diagnosed follicular patients from January 2003 to May 2006 at one of 69 European and American institutions.

Each Relapse of FL Carries a Worse Prognosis

  • FL is an incurable disease characterized by multiple relapses1,4
  • The prognosis for patients with FL worsens with each disease relapse5*
Phase of disease N 5-year PFS
(%)
Median PFS
(years)
First line 2429 56 6.62
Second line 889 28 1.50
Third line 438 18 0.83
Fourth line 229 15 0.69
Fifth line 123 NE 0.63

*According to The National LymphomaCare Study (NLCS), a prospective cohort study of patients diagnosed with follicular lymphoma from March 2004 to March 2007. Median PFS of 6.62 years (95% CI, 6.61-7.2) was reported for patients who received first line therapy (n=2429).

ECOG, Eastern Cooperative Oncology Group; FLIPI, Follicular Lymphoma International Prognostic Index; FLIPI-2, updated version of Follicular Lymphoma International Prognostic Index; LDH, lactate dehydrogenase test; NE, not estimable; OS, overall survival; PFS, progression-free survival.

References: 1. Casulo C, Nastoupil L, Fowler NH, et al. Unmet needs in the first-line treatment of follicular lymphoma. Ann Oncol. 2017;28(9):2094–2106. 2. Barr PM. Counterpoint: can chemotherapy be eliminated in the treatment of follicular lymphoma. Oncology (Williston Park). 32(8):404, 407-409. 3. Shustik J, et al. Follicular non-Hodgkin lymphoma grades 3A and 3B have a similar outcome and appear incurable with anthracycline-based therapy. Ann Oncol. 2011;22: 1164-1169. 4. Luminari S, et al. Follicular lymphoma - treatment and prognostic factors. Rev Bras Hematol Hemoter. 2012;34(1):54-59. 5. Link BK, et al. Second-line and subsequent therapy and outcomes for follicular lymphoma in the United States: data from the observational National LymphoCare Study. Br J Haematol. 2019;184:634-696. 6. Tan D, Hornig SJ, Hoppe RT, et al. Improvements in observed and relative survival in follicular grade 1-2 lymphoma during 4 decades: the Stanford University experience. Blood. 2013; 122(6): 981–987. 7. National Cancer Institute. SEER Cancer Statistics Facts: NHL - Follicular lymphoma. https://seer.cancer.gov/statfacts/html/follicular.html. Accessed January 5, 2019. 8. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for B-Cell Lymphomas V.4.2019. © National Comprehensive Cancer Network, Inc. 2019. All rights reserved. Accessed July 10, 2019. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way. 9. Hoster E, Dreyling M, Klapper W, et al. A new prognostic index (MIPI) for patients with advanced-stage mantle cell lymphoma. Blood. 2008;111(2):558-565. 10. Solal-Céligny P, Roy P, Colombat P, et al. Follicular lymphoma international prognostic index. Blood. 2004;104(5):1258-65. 11. Federico M, Bellei M, Marcheselli L, et al. Follicular lymphoma international prognostic index 2: a new prognostic index for follicular lymphoma developed by the international follicular lymphoma prognostic factor project. J Clin Oncol. 2009;27:4555-4562.