All about adult T-cell leukaemia/lymphoma (ATLL)
Adult T-cell leukaemia/lymphoma (ATLL) is a rare type of non Hodgkin lymphoma that can range from slow growing to aggressive. It affects mature T cells, a type of white blood cell. In a normal healthy person, T cells protect you from infection. In ATLL, T cells grow in an out of control way, and don’t work properly. The main cause of ATLL is the human T-cell lymphotropic virus type 1 (HTLV-1).
ATLL can affect blood and bone marrow in the laukaemia form. It can affect the lymph nodes in the lymphoma form. Although it usually starts in these areas, it can spread to other parts of the body, including organs, bones, and skin.
Symptoms of ATLL
Symptoms vary depending on the type of ATLL. Some symptoms include:
- fatigue
- fever
- night sweats
- unexplained weight loss
- rash, skin lesions
- swelling/lumps in the neck, under arms, or groin
- abdominal discomfort or feeling full
- constipation
- confusion.
Causes of ATLL
ATLL develops in people who have previously been infected with a rare virus called human T-cell lymphotropic virus type 1 (HTLV-1).
HTLV-1 can be passed on through:
- sexual contact
- contact with infected blood
- breastfeeding.
Around 5% of people with HTLV-1 will develop ATLL. There is no way to predict who will develop ATLL.
There are also some other risk factors that may increase your risk of developing ATLL. These include:
- ethnicity – more common in people from Japan, the Caribbean, South and Central America and Africa
- gender – more common in males than females.
Stages of ATLL
ATLL does not have defined stages but is classified as aggressive or indolent. There are four subtypes of ATLL, two aggressive and two indolent.
Types of ATLL
There are two types of adult T-cell leukaemia/lymphoma.
Aggressive adult T-cell leukaemia/lymphoma
Aggressive adult T-cell leukaemia/lymphoma has two subtypes:
- acute
- lymphoma
These aggressive subtypes mainly affect the lymph nodes and are likely to need treatment quickly.
Indolent adult T-cell leukaemia/lymphoma
Indolent adult T-cell leukaemia/lymphoma has two subtypes:
- chronic
- smouldering
These indolent subtypes have symptoms that vary from mild to moderate, and treatment might not be needed right away. Studies show that approximately 50% of people with indolent ATLL eventually progress to aggressive ATLL.
Diagnosis of ATLL
Adult T-cell leukaemia/lymphoma is diagnosed with a number of tests.
If ATLL is in your lymph nodes or you have a lump, you will have a biopsy. This procedure is where a sample of tissue is taken from a swollen lymph node or lump and looked at under a microscope. A skin biopsy may be required if you have a rash or lesions on your skin. This is usually a simple procedure done with a local anaesthetic.
- medical history and physical exam
- blood tests, including human T-lymphotropic virus 1 (HTLV-1) to see if you have the virus
- bone marrow biopsy.
Imaging tests help work out how far the disease has spread and what organs are affected. These tests include:
- computed tomography (CT) scan
- positron emission tomography (PET) scan
Treatment for ATLL
Your treatment for ATLL depends on the subtype and how active the disease is. Common approaches include:
- antiviral therapy
- active monitoring
- chemotherapy
- stem cell transplant.
Your treatment team will recommend the best option for you.
Antiviral therapy may be used to treat some subtypes of ATLL, mostly indolent disease.
If you have a slow growing ATLL with mild or no symptoms, you may not need treatment right away. This is called active monitoring, also called watch and wait. You will have appointments and monitoring with your haematologist and/or general practitioner (GP).
Research shows that, for some people, early treatment does not improve prognosis compared with active monitoring. In some cases, ATLL can be monitored for several years before treatment is needed.
Chemotherapy (chemo) for ATLL is often given in combination with steroids. Chemo kills cancer cells and stops them from growing. It also damages healthy cells and causes side effects. There is information on chemotherapy side effects and how to manage them on our chemotherapy webpage.
Treatment plans for ATLL are shortened to letters, like CHOP. The letters represent the medicines used in that plan. Your haematologist will provide you with information on your plan, including when and how often you will have it.
There are several treatment plans for ATLL. They can be found on the Cancer Institute NSW website.
A bone marrow or blood stem cell transplant is a treatment that restores stem cells after high dose chemotherapy. Your treatment team might suggest a transplant if your ATLL is aggressive, and you go into remission after initial therapy. There are two types of transplants:
- an allogeneic stem cell transplant is when you receive stem cells from a donor, often a blood relative.
- an autologous stem cell transplant is when your own stem cells are collected and then given back to you after high dose chemotherapy.
For more information go to our stem cell transplants webpage.
Clinical trials may be available for some types of ATLL. They often compare new or combination therapies with current treatments, including side effects.
Many are randomised, meaning some patients receive the new treatment, and others receive standard care. Trials help improve future treatments and may offer access to costly new therapies not yet on the PBS in Australia.
If you are considering a clinical trial, your doctor will explain:
- the possible risks and benefits
- how it compares to standard treatment
- what is involved.
You will need to give informed consent before taking part.
Trials are voluntary and run through hospitals and clinics. Read more on our clinical trials webpage.
Survival rate for ATLL
The survival rate of ATLL can range between 11% to 60%, four years from diagnosis. This rate varies depending on your type of ATLL. Survival rate is a population-based measure. Whereas your individual prognosis takes into account factors that can impact survival rate.
Your prognosis is estimated by your haematologist. It is a prediction of the likely course and outcome of your disease. It depends on your type of ATLL and whether it is aggressive or slow growing. Slow growing ATLL usually has a better outlook. The factors considered when discussing your prognosis include:
- your subtype and extent of disease
- your overall health
- your age.
ATLL is difficult to cure, but some people achieve a long-term remission.
Follow-up care for ATLL
Follow up care for ATLL includes regular checkups with your treatment team. This is to check for long term side effects. You will also have tests and scans to make sure it hasn’t come back (relapsed/recurred). You will likely need more treatment if it returns.
Living with ATLL
How ATLL affects your everyday life depends on many factors. It could be that you are managing fatigue, trying to exercise or managing your nutrition. There are some helpful resources and information to guide you on our living well with blood cancer webpage.
Caring for someone with ATLL
We have a range of information and resources that may help when you are caring for someone with ATLL.
Resources for ATLL
Booklets to download:
-
Non-Hodgkin lymphoma (NHL) information booklet
pdf / 1.33 MB
The NHL booklet has been translated into the following languages:
-
Non-Hodgkin lymphoma (NHL) information booklet – Arabic [العربية]
pdf / 10.88 MB
-
Non-Hodgkin lymphoma (NHL) information booklet – Filipino [Pilipino]
pdf / 10.81 MB
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Non-Hodgkin lymphoma (NHL) information booklet – Greek [Ελληνικά]
pdf / 10.89 MB
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Non-Hodgkin lymphoma (NHL) information booklet – Hindi [हिंदी]
pdf / 11.09 MB
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Non-Hodgkin lymphoma (NHL) information booklet – Simplified Chinese [简体中文]
pdf / 11.35 MB
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Non-Hodgkin lymphoma (NHL) information booklet – Traditional Chinese [繁體字]
pdf / 11.77 MB
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Non-Hodgkin lymphoma (NHL) information booklet – Vietnamese [Tiếng Việt]
pdf / 10.90 MB
References
- How I treat adult T-cell leukemia/lymphoma – ScienceDirect
- Adult T-cell leukaemia/lymphoma (ATLL) – Lymphoma Australia
- Adult T-Cell Leukemia/Lymphoma (ATLL): Your Guide | MyLymphomaTeam
- Adult T-Cell Leukemia – StatPearls – NCBI Bookshelf
- Definition of ATLL – NCI Dictionary of Cancer Terms – NCI
- Diagnostic Approaches and Established Treatments for Adult T Cell Leukemia Lymphoma – PMC
- How I treat adult T-cell leukemia/lymphoma | Blood | American Society of Hematology
- New Treatments for Adult T-cell Leukemia/lymphoma – PMC
- Revised Adult T-Cell Leukemia-Lymphoma International Consensus Meeting Report – PMC
- Systematic review of survival outcomes for relapsed or refractory adult T‐cell leukemia‐lymphoma – Nosaka – 2022 – European Journal of Haematology – Wiley Online Library
