Treatment

There is no established consensus on the treatment of follicular lymphoma FL. Since FL is a highly heterogeneous tumor, hematologist/oncologists consider both the disease itself and patient characteristics when developing clinical strategies. Observation may be selected for patients who have asymptomatic disease or for those who have contraindications to available therapies.¹ Current guidelines recommend that patients with stages 1-2 disease receive radiotherapy alone, which reportedly is associated with a 10-year overall survival rate of 60–80% in retrospective studies.¹ Although radiotherapy is considered the standard of care for symptomatic stage 1-2 FL, fewer than a quarter of patients in the National LymphoCare Study were treated with radiotherapy alone (See Reference 2).²

Reference 2

Few prospective or head-to-head comparisons of first-line treatment options for FL have been done.³

Rituximab is broadly credited for improving overall survival in patients with FL in recent years.¹ The anti-CD20 antibody rituximab is administered in combination with chemotherapy regimens or as a monotherapy for the treatment of FL.¹ Addition of rituximab to standard chemotherapy regimens is associated with longer response times and extended overall survival in first-line and relapsed settings based on multiple phase 3 studies.¹

Despite these advancements in disease control, FL is incurable.^2,3 There are several treatment options for patients with relapsed disease, including combination chemotherapy, radioimmunotherapy, and rituximab monotherapy. Additionally, autologous or allogeneic hematopoietic cell transplantation may be appropriate for some patients.¹ Many new agents recently have been investigated for incorporation into FL management for previously untreated or relapsed patients. They include:³

• New anti-CD20 antibodies
• Other antibodies that target B-lineage cells (eg, anti-CD22, anti-CD23)
• Bcl2 inhibitors
• Inhibitors of other oncogenic pathways (eg, PI3K/Akt/mTOR)
• Immunomodulatory drugs

The long-term use of maintenance therapy with rituximab is associated with longer progression-free survival; however, there are higher rates of adverse events.¹ Despite these advances, the majority of patients with a tumor response will experience progression after a treatment response. Patients who experience progression within 2 years of treatment have a poorer prognosis.⁴

Both patients and physicians should remain alert to the subtle signs and symptoms of disease recurrence. Follow-up should include a medical history with particular focus on B symptoms (ie, unexplained weight loss, fever, drenching night sweats, severe itching, and fatigue⁵); a physical examination of the peripheral lymph nodes, liver, and spleen; determination of blood counts and lactate dehydrogenase levels; and occasional imaging.⁶

References

1. Freedman AS. Follicular lymphoma: 2015 update on diagnosis and management. Am J Hematol. 2015;90:1172-1178.
2. Friedberg JW, Taylor MD, Cerhan JR, et al. Follicular lymphoma in the United States: first report of the National LymphoCare Study. J Clin Oncol. 2009;27:1202-1208.
3. Hiddemann W, Cheson BD. How we manage follicular lymphoma. Leukemia. 2014;28:1388-1395.
4. Casulo C, Byrtek M, Dawson KL, et al. Early relapse of follicular lymphoma after rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone defines patients at high risk of death: an analysis from the National LymphoCare Study. J Clin Oncol. 2015;33:2516-2522.
5. Bonander R. Lymphoma B symptoms. LymphomaInfo.net. Available at www.lymphomainfo.net/articles/lymphoma/lymphoma-b-symptoms. Accessed June 3, 2016.
6. Dreyling M, Ghielmini M, Marcus R, et al. Newly diagnosed and relapsed follicular lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014;25(suppl3):iii76-iii82.