Aggressive LGLL
Aggressive LGLL accounts for around 5 in every 100 cases of LGLL (5%). This means there are very low numbers of people with aggressive LGLL.
Aggressive LGLL explained
Aggressive LGLL is very rare so there is limited data on who is affected by it. Some evidence has shown that it mainly affects younger people with an average age of 40, and it is more common in Asian people.
Most cases of aggressive LGLL affect the NK-cells (aggressive NK-LGLL) but there have been some cases that affect T-cells (aggressive T-LGLL).
As with chronic LGLL, we don’t know exactly what causes aggressive LGLL, but it’s not caused by anything you have done.
Researchers have found a link between the Epstein-Barr virus and aggressive LGLL. However, the Epstein-Barr virus is so common that most people will have at some point during their life, and the vast majority never develop blood cancer.
Aggressive LGLL is diagnosed and treated in a similar way to chronic LGLL, but unfortunately aggressive LGLL is harder to treat.
Signs and symptoms of aggressive LGLL
Aggressive LGLL tends to have more severe symptoms than chronic LGLL, and symptoms start and develop quicker.
The main signs of aggressive LGLL include:
- Anaemia (due to low levels of red blood cells), which can cause breathlessness, pale skin and tiredness
- Bruising and bleeding easily (due to low levels of platelets)
- Frequent and persistent infections (due to low levels of white blood cells)
- Unexplained weight loss
- Exhaustion and fatigue that make it hard to continue with daily activities
- Severe night sweats
- A high fever for a prolonged period of time
- A swollen spleen, which might feel like a sense of fullness or discomfort in the upper left abdomen
- A swollen liver
- Enlarged lymph nodes
If you have any of the symptoms above, you should get checked by your GP. Remember that these symptoms can be caused by lots of other things and having them doesn't necessarily mean you have cancer.
Diagnosing aggressive LGLL
Doctors use the same tests for diagnosing both chronic LGLL and aggressive LGLL. This involves taking a sample of your blood or bone marrow and analysing it.
To make a diagnosis of aggressive LGLL, doctors will assess your symptoms and how quickly they came on, and conduct a series of tests:
A full blood count (FBC) will show how many red blood cells, white blood cells and platelets you have, as well as how many abnormal LGLL cells you have. To make a diagnosis of LGLL, doctors will be looking for raised levels of abnormal T-cells or NK-cells for at least six months.
This means checking to see if many of the abnormal cells look identical. This is a sign of leukaemia, because it shows that one specific cell type is cloning itself and multiplying out of control.
All types of cancer happen when something goes wrong in the DNA within cells. In LGLL, a mutation in a gene called STAT5B can indicate aggressive NK-LGLL. These are mutations that happen during your lifetime, they are not mutations that you can inherit or pass down the family.
Certain proteins (known as CD proteins) are found on the surface of lymphocytes. Healthy lymphocytes have typical CD proteins present, but cancer cells normally have an irregular pattern of CD proteins on the surface. Doctors can also use this test to identify what kind of LGLL you have (NK-LGLL or T-LGLL).
Treatment for aggressive LGLL
As with chronic LGLL, aggressive LGLL cannot be cured. Only a very small number of people are diagnosed aggressive LGLL, so there is limited research on what the best treatment options are and there is no standard treatment plan.
Doctors may try a mix of approaches, including:
- Immunosuppressants - drugs which reduce (suppress) the body’s immune response, helping to limit the amount of abnormal LGLL cells being made in the body.
- Immunotherapy - drugs which help your own immune system to identify and attack cancer cells.
- Chemotherapy – drugs that destroy cells that multiply very quickly, like cancer cells.
- Splenectomy – having your spleen removed if it is very enlarged and causing problems.
So far, research has told us that people with aggressive LGLL do not generally respond well to treatment and the overall prognosis is poor. However, a small number of people with aggressive LGLL have reached remission (where there are no signs of active cancer).
A chemotherapy called L-asparaginase has shown some promising results in people with aggressive NK-LGLL and is considered the first treatment choice for NK-LGLL.
For people with aggressive LGLL that does respond to initial treatment, doctors will likely recommend going on to have a donor stem cell transplant. A stem cell transplant is a very intensive treatment, but it can offer the chance of remission in some people. If your medical team think a transplant might be an option for you, they will talk you through it. We have more detailed information about stem cell transplants.
Prognosis for aggressive LGLL
The overall prognosis for aggressive forms of LGLL is poor. Aggressive LGLL develops and progresses quickly, and is often resistant to treatment. Unfortunately, the average time that people survive with aggressive LGLL is a few months. However, this is an average taken across multiple people with LGLL, and within this group some people have achieved remission.
As there is limited data available on aggressive LGLL, it is difficult to predict how any one person will respond to treatment. Some people with aggressive LGLL have been successfully treated, and research is continuing to further our knowledge and understanding of LGLL.
If you are diagnosed with aggressive LGLL, your team will talk you through what to expect from treatment. If treatment doesn’t work, your team may discuss palliative care with you. This is care to help you feel as comfortable as possible. This also includes talking about your wishes for the future, helping you and your loved ones come to terms with your experience.
Worried or have questions about prognosis?
We can talk you through it and support you to have conversations with your doctor.