Follicular lymphoma

Follicular lymphoma is a B-cell lymphoma. Follicular describes how the lymphoma cells group together in clusters in a lymph node or other tissues.

Follicular lymphoma is the 2nd most common type of non-Hodgkin lymphoma (NHL). It usually occurs in people 50 years of age or older, and the average age at diagnosis is 59. It is slightly more common in women than in men.

Most people are diagnosed with stage 3 or stage 4 follicular lymphoma. It often spreads to the bone marrow and spleen, but it usually doesn’t affect organs and tissues other than the lymph nodes.

Sometimes follicular lymphomas can change into diffuse large B-cell lymphoma (DLBCL). DLBCL is a fast-growing (aggressive) type of NHL.

Follicular lymphoma may not cause any symptoms. Sometimes lymph nodes in different parts of the body can be larger than normal.

The healthcare team uses the Follicular Lymphoma International Prognostic Index (FLIPI) to help them assess people with this disease and plan treatment. Those with good prognostic factors are likely to respond well to treatment. There is a greater chance that follicular lymphoma will come back (recur) after treatment when someone has poor prognostic factors.

The healthcare team gives 1 point for each poor prognostic factor. They add up the points for a score of 0 to 5. People with good prognostic factors and a lower score have a better prognosis than those with 3 or more poor prognostic factors and higher scores.

There are many treatment options for follicular lymphoma. This type of NHL often comes back (recurs) after treatment, but it usually responds to more treatment. People often reach remission again after more treatment, but the remissions usually become shorter with every course of treatment.

External beam radiation therapy is used to treat lymph node areas affected by stage 1 or stage 2 follicular lymphoma. This may be referred to as involved field radiation therapy (IFRT).

Follicular lymphoma is usually more advanced (stage 3 or 4) and has usually spread to more than one lymph node area in the body when it is diagnosed. Low-dose external beam radiation therapy may be used to help relieve symptoms when follicular lymphoma is more advanced.

Watchful waiting (also called active surveillance) may be offered for follicular lymphoma because it develops slowly and may not need to be treated right away. The healthcare team carefully monitors the person with follicular lymphoma and starts treatment when symptoms appear or there are signs that the disease is progressing more quickly.

Chemotherapy may be offered for follicular lymphoma, especially if it has spread to several groups of lymph nodes or other organs and it is causing symptoms. A single drug or a combination of drugs may be used. Chemotherapy is often combined with a targeted therapy drug.

Single drugs that may be given include:

• fludarabine (Fludara)
• bendamustine (Treanda)
• chlorambucil (Leukeran)

Combinations of drugs given for follicular lymphoma include:

• CHOP – cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone
• R-CHOP – CHOP with rituximab (Rituxan)
• CVP – cyclophosphamide, vincristine and prednisone
• R-CVP – CVP with rituximab
• FND – fludarabine, mitoxantrone (Novantrone) and dexamethasone (Decadron, Dexasone)
• BR – bendamustine and rituximab

Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.

Rituximab is a targeted therapy drug used to treat follicular lymphoma. It can be used alone or with chemotherapy. It may also be used as maintenance therapy after chemotherapy.

Other targeted therapies that may be used when follicular lymphoma comes back after treatment (recurs) or doesn’t respond to treatment (called refractory disease) are:

• ibritumomab (Zevalin)
• idelalisib (Zydelig)
• obinutuzumab (Gazyva) with bendamustine

Ibritumomab is a type of radioimmunotherapy. This treatment attaches a radioactive material to the targeted therapy drug. The drug attaches to the lymphoma cells so the radiation is delivered directly to them, which may mean fewer or less severe side effects.

A stem cell transplant may be an option for some people if follicular lymphoma comes back (recurs) after treatment or doesn’t respond to treatment (refractory disease).