Therapies of Relapsed and Refractory Follicular Lymphoma Updated

According to a recent article, Connie Lee Batlevi, MD, PhD, who is a medical oncologist, recently shared her experiences and knowledge pertaining to the treatment of follicular lymphoma.

Follicular Lymphoma

Follicular lymphoma is a form of non-Hodgkin’s lymphoma, which is a cancer of the lymphatic system. The lymphatic system is a part of the immune system and a complex series of thin vessels, like blood vessels, that run throughout the body. Lymph vessels carry a clear liquid called lymph that contains a type of white blood cell (lymphocyte). The lymphatic system also contains lymph nodes, the spleen, thymus, and tonsils. Follicular lymphoma usually occurs in many lymph nodes throughout the body, as well as in bone marrow.

Follicular lymphoma is a slow-growing (indolent) cancer, although in rare cases it may grow quickly. About one in five lymphomas in the U.S. is a follicular lymphoma. On average, it is most likely to occur in people at around age 60. It is very rare in young people.


Since this cancer is slow-growing, patients will often not show obvious symptoms at the time of diagnosis. Symptoms include:

  • Swollen lymph nodes in the neck, abdomen, underarms, and/or groin
  • Fatigue
  • Shortness of breath
  • Weight loss
  • Night sweats
  • Fever

Some patients may eventually develop a transformed lymphoma, which is more aggressive than a follicular lymphoma.

Choosing Therapy After Relapse

Patients with follicular lymphoma have a high risk of disease relapse, especially since it is noncurable. After a patient has a first relapse, there are many factors that affect what treatment will work best. These factors include a patient’s functional status, disease characteristics (after relapse), disease volume on imaging, laboratory findings, metabolic activity on PET scan, and genetic and pathologic assessments.

As Batlevi pointed out, it is extremely important for a patient to get a biopsy. This is vital in the disease assessment and thus in choosing the right treatment.

Treatment is also dependent on the state of the patient. For example, if a patient is frail, they will not be able to withstand some treatments. Furthermore, if patients are unable to follow a meticulous schedule for treatment, that can affect the treatment chosen for them.

Batlevi uses a set of guidelines in order to help her decide the best treatment for every patient. The guidelines she chooses to use are the Groupe d’Etudes des Lymphomes Folliculaires.

Third-line and Beyond Therapy Options

The third-line treatment, or treatment that is given after initial treatment and subsequent treatments don’t work or stop working, has several options. Some options include chemotherapy, while others do not.

Chemotherapy options consists of autologous stem cell transplants which feature high doses of chemotherapy. Non-chemotherapy options are comprised of PI3K inhibitors, EZH2 inhibitors, immune-based therapies, and allogenic stem cell transplant.


For patients with follicular lymphoma, it is specifically important to undergo molecular testing to help choose the most effective therapy. This can show, for example, if a treatment featuring the recently approved agent tazemetostat is the best option.

Tazemetostat is a drug that is given orally twice a day. It is used for patients with EZH2-postive relapsed or refractory follicular lymphoma, and patients who have no other satisfactory alternative treatment options. This treatment is particularly beneficial since it quite safe. Most patients tolerate the drug well – less than 10% require dose reductions, and fewer than 5% have to stop the treatment.

Hope For The Future

The approval of the first EZH2 inhibitor for the for the treatment of follicular lymphoma is a good sign for the future of treatment options, says Batlevi. Targeted agents as treatment options will likely continue to be researched.

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