Chronic lymphocytic leukaemia - Treating chronic lymphocytic leukaemia

Treatment for chronic lymphocytic leukaemia will depend on what stage the condition is at when it's diagnosed.

Other factors will also be considered, such as your age and overall level of health and fitness.

Staging

Doctors use stages to describe how far leukaemia has developed. In the UK, the Binet staging system is used for chronic lymphocytic leukaemia. It has three stages:

• Stage A – where you have fewer than three areas of enlarged lymph nodes and a high white blood cell count
• Stage B – where you have more than three areas of enlarged lymph nodes and a high white blood cell count
• Stage C – where you have enlarged lymph nodes or spleen, a high white blood cell count and a low red blood cell or platelet count

An area of lymph nodes refers to lymph nodes in one area of your body, such as in your neck, under you arms or in your groin.

If the lymph nodes on both sides of your body are swollen – under both arms, for example – this counts as one area.

Treating early stage chronic lymphocytic leukaemia

Treatment may not be needed if you don't have any symptoms when you're diagnosed with chronic lymphocytic leukaemia.

The condition often develops very slowly, and if you don't have any symptoms there is no advantage in starting treatment early.

In such cases, a policy of "watchful waiting" is usually recommended, which involves regular visits to your doctor and blood tests, so that the condition's progress can be carefully monitored.

Treatment for chronic lymphocytic leukaemia will usually only be recommended if you have:

• symptoms, such as severe tiredness and weight loss 
• enlarged lymph nodes
• bone marrow failure 
• rapidly rising numbers of lymphocytes (white blood cells) in your bloodstream

Chemotherapy (see below) is usually the first treatment that's recommended. Radiotherapy can also be used to shrink enlarged lymph nodes, and surgery can be used to remove a swollen spleen.

Treating symptomatic chronic lymphocytic leukaemia

As chronic lymphocytic leukaemia often develops very slowly, the timescales for treatment vary considerably.

Some people don't experience symptoms for a long time and may not need treatment for many years, possibly decades.

For others, particularly those with certain genetic abnormalities, the condition may progress much faster (within a few months).

If you have later-stage chronic lymphocytic leukaemia (stage B or C), your haematologist (blood specialist) will probably recommend chemotherapy.

Chemotherapy

The standard treatment programme for chronic lymphocytic leukaemia is a combination of three medications: fludarabine, cyclophosphamide and rituximab (FCR).

Fludarabine and cyclophosphamide are usually given in tablet form and taken for five days every 28 days. Rituximab is given intravenously (directly into a vein) over the course of several hours.

You can read the NICE guidance about fludarabine for treating chronic lymphocytic leukaemia.

Most people benefit from FCR. Although it won't cure the condition, it usually leads to remission – where there's no sign of leukaemia and you don't have any symptoms. It can last for many months or several years.

After a period of remission, the leukaemia can return and you may begin to experience symptoms again. This is known as a relapse. If chronic lymphocytic leukaemia returns, you may need to have more chemotherapy and biological therapy.

Chemotherapy can weaken your immune system, which is known as being immunocompromised. This can be serious, as it can cause infections to develop.

You may be prescribed a course of antibiotics to take during your chemotherapy treatment to help prevent infections.

Read more about the complications of chronic lymphocytic leukaemia.

Rituximab

Rituximab is a monoclonal antibody, which are genetically engineered in a laboratory.

Rituximab works by sticking to a protein found on the surface of  the lymphocytes (cancerous white blood cells). The immune system then targets and destroys the lymphocytes.

Rituximab is commonly used in combination with other chemotherapy medication, such as fludarabine and cyclophosphamide (see above).

It's given intravenously (directly into a vein) over the course of several hours. This is known as an infusion.

Common side effects of rituximab include flu-like symptoms, such as feeling sick, headache, a high temperature and chills. You may also have an itchy rash in the area where the medication enters the vein.

Most side effects of rituximab occur within 24 hours of treatment starting for the first time. You will be closely monitored once your treatment begins.

If you start to experience an adverse reaction, such as shortness of breath or chest pain, anti-allergy medicines, such as corticosteroids, can be used to help relieve your symptoms.

Other chemotherapy options

Alemtuzumab

Alemtuzumab is like rituximab – a medication called a monoclonal antibody that recognises certain proteins on the surface of lymphocytes, attaches to them and helps destroy them. Alemtuzumab and rituximab recognise different surface proteins.

Alemtuzumab may be considered if your leukaemia has stopped responding to chemotherapy, or has returned following chemotherapy treatment.

Like rituximab, alemtuzumab is administered intravenously. It can also be given by injection.

The main side effect of alemtuzumab is that it can weaken your immune system and make you vulnerable to infection. You will therefore be prescribed antibiotics while taking it.

Bendamustine

Bendamustine is a fairly new medication for treating chronic lymphocytic leukaemia.

The National Institute for Health and Care Excellence (NICE) recommends bendamustine as a treatment option for people with advanced chronic lymphocytic leukaemia (stage B or C) who are unable to tolerate fludarabine.

Bendamustine is given by infusion, twice a week every four weeks. This cycle of treatment may be repeated up to six times. The most serious side effect of bendamustine is a weakened immune system. It may be given in combination with rituximab.

You can read the NICE guidance about bendamustine for treating chronic lymphocytic leukaemia.

Chlorambucil

Chlorambucil is a chemotherapy medication that is given in tablet form usually for seven days, once a month for six months.

It's sometimes given in combination with rituximab. It can have side effects, such as low blood counts and infections, but is generally well tolerated. It's sometimes used in people who aren't fit enough to receive FCR or bendamustine.

Ofatumumab

Ofatumumab is a monoclonal antibody that is similar to rituximab. It's sometimes used in people with chronic lymphocytic leukaemia that's relapsed after receiving fludarabine and alemtuzumab, or in those who aren't fit enough to receive these medications.

It's given as an infusion, and the main side effects are allergic reactions and low blood counts.

Bone marrow and stem cell transplants

Bone marrow or stem cell transplants are sometimes used to treat chronic lymphocytic leukaemia.

This type of intensive treatment aims to get rid of chronic lymphocytic leukaemia completely, or control it for longer periods.

Before having a bone marrow or stem cell transplant, you will have a number of tests to check you're fit enough to have this type of intensive treatment.

You'll also be given aggressive, high-dose chemotherapy and radiotherapy to destroy any cancerous cells in your body and put the leukaemia into remission. This can place significant strain on your body and can cause additional side effects.

Bone marrow and stem cell transplants have better outcomes when the donor has the same tissue type as the person receiving the donation. Therefore, the best donor is usually a brother or sister with the same tissue type.

Due to these issues, transplants are usually only successful when they're carried out in children and young people, or older people in good health, and there's a brother or sister who's able to provide a donation.

In many cases of chronic lymphocytic leukaemia, the potential risks of transplantation far outweigh any benefit. For example, the chances of an elderly person with advanced chronic leukaemia surviving a bone marrow transplant can be as low as one in five.

Read more about bone marrow transplants.

Deciding against treatment

As many of the treatments described in this section have unpleasant side effects that can affect your quality of life, you may decide against having a particular type of treatment.

This is entirely your decision and your treatment team will respect any decision you make. You won't be rushed into making a decision about your treatment, and before making a decision you can talk to your doctor, partner, family and friends.

Pain relief and nursing care will be made available as and when you need it.