Disease characteristics, treatment patterns, and outcomes of follicular lymphoma in patients 40 years of age and younger: an analysis from the National Lymphocare Study†

Abstract

Background: Follicular lymphoma (FL) is the most common indolent non-Hodgkin lymphoma, with median age at diagnosis in the seventh decade. FL in young adults (YAs), defined as diagnosis at ≤40 years, is uncommon. No standard approaches exist guiding the treatment of YA FL, and little is known about their disease characteristics and outcomes. To gain further insights into YA FL, we analyzed the National LymphoCare Study (NLCS) to describe characteristics, initial treatments, and outcomes in this population versus patients aged >40 years.

Patients and methods: Using the NLCS database, we stratified FL patients by age: 18-40 (YA), 41-60, 61-70, 71-80, and >80 years. Survival probability was estimated using Kaplan-Meier methodology. We examined associations between age and survival using hazard ratios and 95% confidence intervals (CIs) from multivariable Cox models.

Results: Of 2652 eligible FL patients in the NLCS, 164 (6%) were YAs. Of YA patients, 69% had advanced disease, 80% had low-grade histology, and 50% had good-risk disease according to the Follicular Lymphoma International Prognostic Index (FLIPI). Nineteen percent underwent observation, 12% received rituximab monotherapy, and 46% received chemoimmunotherapy [in 59% of these: R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone)]. With a median follow-up of 8 years, overall survival (OS) at 2, 5, and 8 years was 98% (95% CI 93-99), 94% (95% CI 89-97), and 90% (95% CI 83-94), respectively. Median progression-free survival (PFS) was 7.3 years (95% CI 5.6-not reached).

Conclusions: In one of the largest cohorts of YA FL patients treated in the rituximab era, disease characteristics and outcomes were similar to patients aged 41-60 years, with favorable OS and PFS in YAs. Longer-term outcomes and YA-specific survivorship concerns should be considered when defining management. These data may not support the need for more aggressive therapies in YA FL.

Clinical trial number: Roche/Genentech ML01377 (U2963n).

Keywords: follicular lymphoma; outcomes; survival; treatment; young adults.

Figure 1.

PFS for (A) all active treatment (watchful waiting excluded), (B) rituximab plus chemotherapy, and (C) rituximab monotherapy, and OS for (D) all active treatment (watchful waiting excluded), (E) rituximab plus chemotherapy, and (F) rituximab monotherapy, by age group and active first-line treatment. PFS and OS curves were estimated from the Cox proportional-hazards model with adjustment for FLIPI components, specifically, hemoglobin ≥12 g/dl, normal lactate dehydrogenase, <5 nodal sites, and stage I or II. FLIPI, Follicular Lymphoma International Prognostic Index; OS, overall survival; PFS, progression-free survival.