What is indolent lymphoma?

Indolent lymphoma is a type of non-Hodgkin’s lymphoma (NHL) that grows and spreads slowly. An indolent lymphoma usually won’t have any symptoms.

Lymphoma is a type of cancer of the white blood cells of the lymphatic, or immune, system. Hodgkin’s lymphoma and non-Hodgkin’s lymphoma differ based on the type of cell it attacks.

Life expectancy for this disease

The average age of those who are diagnosed with indolent lymphoma is about 60. It affects both men and women. The average life expectancy after diagnosis is approximately 12 to 14 years.

Indolent lymphomas are about 40 percent of all NHLs combined in the United States.

What are the symptoms?

Because indolent lymphoma is slow growing and slow to spread, you may not have any noticeable symptoms. However, possible symptoms are those that are common to all non-Hodgkin’s lymphomas. These common NHL symptoms may include:

  • swelling of one or more lymph nodes, which usually isn’t painful
  • fever that isn’t explained by another illness
  • unintended weight loss
  • loss of appetite
  • severe night sweats
  • pain in your chest or abdomen
  • severe fatigue that doesn’t go away with rest
  • feeling full or bloated all the time
  • spleen or liver become enlarged
  • skin that feels itchy
  • bumps on your skin or a rash

Types of indolent lymphoma

There are several subtypes of indolent lymphoma. These include:

Follicular lymphoma

Follicular lymphoma is the second most common subtype of indolent lymphoma. It makes up 20 to 30 percent of all NHLs.

It’s extremely slow growing, and the average age at diagnosis is 50. Follicular lymphoma is known as an elderly lymphoma because your risk increases when you exceed age 75.

In some cases, follicular lymphoma can progress to become diffuse large B-cell lymphoma.

Cutaneous T-cell lymphomas (CTCLs)

CTCLs is a group of NHLs that usually start in the skin and then spread to include your blood, lymph nodes, or other organs.

As a CTCL progresses, the name of the lymphoma changes depending on where it has spread. Mycosis fungoides is the most noticeable type of CTCL because it affects the skin. When a CTCL moves to include the blood, it’s called Sézary syndrome.

Lymphoplasmacytic lymphoma and Waldenström macroglobulinemia

Both of these subtypes start in a B lymphocyte, a specific type of white blood cell. Both can become advanced. In the advanced stage, they can include your gastrointestinal tract, your lungs, and other organs

Chronic lymphocytic leukemia (CLL) and small cell lymphocytic lymphoma (SLL)

These subtypes of indolent lymphoma are similar in both symptoms and the average age at diagnosis, which is 65 years. The difference is that SLL primarily affects lymphoid tissue and the lymph nodes. CLL primarily affects the bone marrow and the blood. However, CLL can spread into the lymph nodes as well.

Marginal zone lymphoma

This subtype of indolent lymphoma begins in the B lymphocytes in an area called the marginal zone. This disease tends to remain localized in that marginal zone. Marginal zone lymphoma in turn has its own subtypes, which are determined based on where they’re located in your body.

Causes of indolent lymphoma

It’s currently unknown what causes any NHL including indolent lymphoma. However, some types may be caused by an infection. There are also no known risk factors related to NHL. However, advanced age may possibly be a risk factor for most people with indolent lymphoma.

Treatment options

Several treatment options are used for indolent lymphoma. The stage or severity of the disease will determine the amount, frequency, or combination of these treatments. Your doctor will explain your treatment options and determine which treatment will work best for the particular disease and how advanced it is. Treatments may be changed or added to depending on the effect they have.

Treatment options include the following:

Watchful waiting

Your doctor may also call this active surveillance. Watchful waiting is used when you don’t have any symptoms. Because indolent lymphoma is so slow growing, it may not need to be treated for a long time. Your doctor uses watchful waiting to closely monitor the disease with regular testing until the lymphoma needs to be treated.

Radiation therapy

External beam radiation therapy is the best treatment if you have only one lymph node or a few affected. It’s used to target only the area that is affected.

Chemotherapy

This treatment is used if radiation therapy doesn’t work or there is too large an area to target. Your doctor may give you only one chemotherapy medication or a combination of two or more.

The chemotherapy medications most commonly given individually are fludarabine (Fludara), chlorambucil (Leukeran), and bendamustine (Bendeka).

The combination chemotherapy drugs that are most commonly used are:

  • CHOP, or cyclophosphamide, doxorubicin (Doxil), vincristine (Oncovin), and prednisone (Rayos)
  • R-CHOP, which is CHOP with the addition of rituximab (Rituxan)
  • CVP, or cyclophosphamide, vincristine, and prednisone
  • R-CVP, which is CVP with the addition of rituximab

Targeted therapy

Rituximab is the medication used for targeted therapy, usually in combination with chemotherapy medications. It’s only used if you have a B-cell lymphoma.

Stem cell transplant

Your doctor may recommend this treatment if you have a relapse or the indolent lymphoma returns after remission and other treatments don’t work.

Clinical trials

You can talk to your doctor to see if you’re eligible for any clinical trials. Clinical trials are treatments that are still in the testing phase and aren’t yet released for general use. Clinical trials are usually an option only if your disease returns after remission and other treatments aren’t helping.

How is it diagnosed?

Often indolent lymphoma is initially found during a routine medical exam (for example, a complete physical exam by your doctor) because you likely won’t have any symptoms.

However, once discovered, more thorough diagnostic testing is required to determine the type and stage of your disease. Some of these diagnostic tests may include the following:

The tests you need depend on the area indolent lymphoma is affecting. The results of each test may require you to have additional tests. Talk with your doctor about all the testing options and procedures.

Complications of this disease

If you have a later stage of follicular lymphoma, it can be more difficult to treat. You’ll will have a higher chance of relapse after you achieve remission.

Hyperviscosity syndrome can be a complication if you have either lymphoplasmacytic lymphoma or Waldenström macroglobulinemia. This syndrome occurs when cancer cells create a protein that isn’t normal. This abnormal protein can lead to thickening of the blood. Thickened blood in turn inhibits blood flow through the body.

Chemotherapy medications can have complications if they’re used as part of your treatment. You should discuss both the possible complications and benefits of any treatment option to determine the best treatment for you.

Outlook for indolent lymphoma

If you’re diagnosed with indolent lymphoma, you should work with a medical professional who has experience treating this type of lymphoma. This type of doctor is called hematologist-oncologist. Your primary doctor or insurance carrier should be able to refer you to one of these specialists.

Indolent lymphoma can’t always be cured. However, with early diagnosis and proper treatment, it may go into remission. Lymphoma that does go into remission may eventually be cured, but that isn’t always the case. An individual’s outlook is dependent on the severity and type of their lymphoma.