Treatment options for hairy cell leukaemia

A team of health professionals decides if you need treatment straight away. They also decide what treatment you have.

The main treatment for hairy cell leukaemia is chemotherapy. Other treatments include a targeted drug such as rituximab.

Deciding which treatment you need

A team of doctors, and other professionals discuss the best treatment and care for you. They are the multidisciplinary team (MDT). Your MDT might include:

• a haematologist - a doctor specialising in blood cancers
• a haemopathologist – a doctor who examines bone marrow or lymph node biopsies
• a radiologist – a doctor specialising in reporting x-rays and scans
• a specialist haematology nurse – also called a clinical nurse specialist (CNS)
• a palliative care doctor - a doctor specialising in controlling cancer symptoms

You may not need treatment straight away. Your team decides whether you need treatment depending on your symptoms.

Your doctor will talk to you about your treatment options. They will discuss the benefits and the possible side effects with you.

The treatment you have depends on:

• your symptoms and whether you have an infection
• your general health and level of fitness

The main treatments

You are likely to have chemotherapy as your main treatment. Other treatments include targeted and immunotherapy drugs. Rarely you might have surgery to remove your spleen.  

Chemotherapy

Chemotherapy uses anti cancer (cytotoxic) drugs to destroy cancer cells. The drugs circulate throughout the body in your bloodstream. Cladribine and pentostatin are examples of chemotherapy drugs for hairy cell leukaemia.

You usually have one type of chemotherapy on its own.

Targeted and immunotherapy drugs

Targeted cancer drugs work by targeting the differences in cancer cells that help them to grow and survive. Other drugs help the immune system to attack the cancer. They are called immunotherapies.

Some targeted drugs are also a type of immunotherapy. These are called a monoclonal antibodies (MABs). Rituximab is an example of a MAB for hairy cell leukaemia. You usually have it in combination with chemotherapy.

Interferon is a type of immunotherapy. Doctors don’t often use interferon for hairy cell leukaemia any more. This is because newer chemotherapy drugs work better.

Surgery

Surgery is not a common treatment for hairy cell leukaemia. But rarely, the doctor might suggest an operation to remove the spleen (splenectomy). Removing the spleen can help with some of the symptoms caused by an enlarged spleen.

If you don't have symptoms

Your doctor might not offer you treatment if you don't have any symptoms at diagnosis. Instead, they keep a close eye on you. You have careful monitoring with tests and check ups every 3 to 6 months. You might hear this called watch and wait.

Some people don’t need treatment for years. You begin treatment if your blood count changes or if you develop symptoms.

If you have symptoms

Most people need to start treatment soon after diagnosis. You usually start treatment straight away if you have symptoms. Symptoms include low blood counts, an enlarged spleen, or repeated infections. The first treatment you have is called first line treatment.

The aim of treatment is to get rid of the leukaemia cells (remission). With hairy cell leukaemia, remission can last for years.

You usually have a chemotherapy drug. The most common drugs are:

• cladribine
• pentostatin

If you need further treatment

You might need further treatment if:

• tests show that your hairy cell leukaemia has not responded to first line treatment
• hairy cell leukaemia comes back (relapses) after a period of time

The next lot of treatment you have is called second line treatment. You can have a further remission with more treatment. The remissions tend to get shorter, the more treatment you have.

There are different options for second line treatment. Your treatment depends on:

• what first line treatment you had
• how long you have been in remission
• what clinical trials are available
• your general health and symptoms

Treatment options include:

• the same chemotherapy drug (cladribine or pentostatin) as you had before - you have it on its own or combined with rituximab
• the other chemotherapy drug (cladribine or pentostatin), usually combined with rituximab
• clinical trials looking at different targeted drugs

Most people respond well to treatment and have another remission. But there are other treatment options if you relapse again. These include:

• a different chemotherapy (such a bendamustine) combined with rituximab
• clinical trials looking at different targeted drugs

Other treatments

Other treatments you might have for hairy cell leukaemia include:

• surgery to remove your spleen
• supportive treatments such as antibiotics, blood products or steroids

Clinical trials

Your doctor might ask if you’d like to take part in a clinical trial. Doctors and researchers do trials to make existing treatments better and develop new treatments.