Chronic LGLL treatment and side effects

Your hospital team will plan your treatment and consider factors like your symptoms, blood counts, and general health. Some people won't even need any treatment for LGLL at first, if it is not causing them any symptoms. This is known as active monitoring.

It might take some time to understand your condition, and getting diagnosed can feel overwhelming. Take your time to process the information, and when you are ready, your team will be there to support you with any questions you might have.

Many people who are diagnosed with chronic LGLL do not need treatment straight away if they have no symptoms. This is called active monitoring.

If your doctors recommend an active monitoring approach, you will have regular tests to check whether anything is progressing. If you do begin to get symptoms, your doctors can start you on treatment. Some people end up not needing treatment at all if there are no signs of the leukaemia progressing, but most people with LGLL will end up needing treatment at some point.

For those who don’t have treatment straight away, it can be challenging coming to terms with the uncertainty and emotional impact of having cancer but not having treatment. Many find it helpful to connect with others in a similar position through our online community forum.

We have more information about what to expect from active monitoring here.

If you have symptoms of LGLL or low levels of healthy blood cells at diagnosis, or if you develop symptoms while on active monitoring, treatment will be needed.

The first line of treatment for people with chronic T-LGLL or chronic NK-LGLL is usually immunosuppressants.

Immunosuppressants are drugs which help to control the body’s immune response. Normally, when your body detects a germ, bacteria or virus,, your immune system produces an inflammatory response to help fight off the invader. In LGLL, the immune system is in an abnormal inflammatory state, firing signals even when there is no invader. This causes more and more lymphocytes to be produced, until eventually there is no room for healthy blood cells in the body.

Immunosuppressant drugs work by reducing this abnormal immune response, helping to limit the amount of LGLL cells in the body. The main immunosuppressant drugs used are:

• Low-dose methotrexate – this is the most common treatment option for LGLL.
• Cyclophosphamide – this may be used if methotrexate is not effective, or if you have particularly low levels of neutrophils (another type of white blood cell)
• Cyclosporin A (also spelled ciclosporin) – this is another treatment option for LGLL and is often preferred for people who have anaemia along with their LGLL, especially if they have pure red cell aplasia (a specific complication of LGLL).

The type of immunosuppressant drug you have will depend on your own circumstances. These drugs are normally taken by mouth. Your treatment team will advise you on how many to take, and how long to take them for.

How long you’ll take immunosuppressants for will depend on how your body responds to the treatment. Doctors usually wait at least 4 months while you take the drug before assessing your response.

You are likely to have some supportive treatments alongside your treatment.

While active treatment (like immunosuppressants) aims to treat the LGLL, supportive treatment does not target the cancer but helps to boost the number of healthy blood cells you have. This can improve your symptoms and help your body cope with the LGLL. Throughout your treatment you might have:

• Blood transfusions, to improve your counts of red blood cells and platelets
• Growth factor injections (G-CSF), which help your body generate more healthy blood cells like neutrophils (a key type of white blood cell).

Doctors will assess your response to first-line treatment after roughly 4 to 6 months. They will do more tests to see whether your blood counts have improved and the number of LGLL cells has gone down.

If there are no signs of active LGLL after treatment, then you are in remission.

Many people remain in remission for a very long time and can live a relatively normal life. When you are in remission, you will have regular check-ups and appointments to see:

• how you are recovering from treatment (both physically and mentally)
• if you have any long-term side effects
• if you are showing any symptoms of LGLL coming back.

This is the same as being back on active monitoring.

You do not have to wait until a follow-up appointment to talk to a doctor if you are concerned about anything though. If anything worries you in between appointments, contact your blood cancer team – you should have a contact number or email for them.

Sometimes, first-line treatment works initially, but then later the LGLL begins to grow back again. This is called a relapse.

For some people, first-line treatment does not work at all. This is called refractory LGLL.

If you have relapsed or refractory LGLL, there are different treatments to try – these are called are second-line treatments.

If both first-line and second-line treatments are not successful at getting you into remission, doctors may look into clinical trials for you.

Clinical trials offer the chance to have one of the latest treatment options and to be part of discovering better ways to treat LGLL.

If you enter a clinical trial, your doctor will explain what this will involve and give you more information on the treatment being trialled. You can also ask your team any questions about the trial.

You do not have to consent to taking part in a clinical trial. If you don’t want to, you’ll be offered the best available standard treatment.

If you want to learn more about the clinical trials available to you or have any questions about being involved in a clinical trial, see our clinical trials information, including our Clinical Trials Support Service.

Treatments such as immunosuppressants, immunotherapy and combined chemotherapy aim to destroy the cancer cells within your body. However, these drugs can also impact your healthy cells too and prevent how well the immune system functions. This is what causes side effects.

Side effects can vary a lot from person to person, depending on what drug and dose is used, and any other medical conditions you might have. Even two people on the same treatment can have very different experiences.

There are lots of possible side effects, but remember you are very unlikely to have them all.

Some of the more common short-term side effects seen with LGLL drugs are listed below.

Remember, there are things that can be done about most side effects. Tell your healthcare team about any you experience – there is almost always something they can do to reduce it.

Treatments for cancer can have longer-term side effects such as nerve damage, bone and joint pain, and changes to how the thyroid gland works.

Some types of treatment can also impact your ability to get pregnant or make someone pregnant (fertility problems). If this is something that worries you, talk to your hospital team. They can discuss what to do if your treatment affects your fertility, and tell you whether your fertility is likely to come back afterwards.

Side effects vary from person to person, and your team will talk you through what short-term and long-term side effects to watch out for. You are unlikely to experience all the side effects listed above, but side effects can be unpleasant and might impact your daily life.

There are some things you and your team can do to help manage the side effects of treatment:

• You will probably be given growth factors (called G-CSF) – these are injections that help boost your immune system and reduce or prevent frequent infections. They help your body generate more healthy white blood cells.
• You can also be prescribed antiemetic (anti-sickness) medication, which can help reduce vomiting and nausea.
• You can make changes to your diet and lifestyle which can help reduce your risk of infections, as well as improve symptoms of fatigue (tiredness).
• Some people find that learning certain mindfulness and relaxation techniques can improve well-being, as well as help to cope with physical symptoms.

Your healthcare team will be able to provide you with guidance around what might be best for you and point you to any more sources of support.