Cutaneous T-cell LymphomaYour guide to best cancer care

It can be overwhelming after being told that you have or may have cutaneous T-cell lymphoma (CTCL). A lot can happen quickly, and you might have lots of questions, like 'What happens next after a diagnosis?'

This support resource can help to guide you, your family, and your friends through this experience. Other international resources are available online but may discuss approaches to testing and treatment that are not standard of care in Australia.

The two most common types of CTCL include mycosis fungoides and Sézary syndrome. CTCL is often a slow progressing disease and generally has an excellent prognosis – especially if identified in the early stages.

Information and support

If you need additional information or would like support, you can visit the non-hodgkin lymphoma page or call Cancer Council on 13 11 20 to talk to an experienced healthcare professional.

To speak with an experienced healthcare professional who can provide you with disease-specific information, answer your questions, talk through your concerns, and connect you to blood cancer support groups, call 1800 620 420 or visit the Leukaemia Foundation page. The team can also help with practical concerns such as accommodation close to treatment, transport to appointments, and financial assistance.

Lymphoma Australia nurses can help patients and their families understand their diagnosis and treatment options, navigate the lymphoma journey, and connect patients with others and appropriate support networks. Call 1800 953 081 or visit the Lymphoma Australia page for education, support, and awareness for lymphoma and CTCL.

More information is available in the ‘Resource’ section of the optimal care pathway for cutaneous T-cell lymphoma.

Information, resources and support for carers and loved ones are available on the Leukaemia Foundation website and on the Cancer Council website.

If you need a translator, call TIS on 13 14 50 or visit the TIS National website.

Symptoms

Your general practitioner (GP), other primary care physician, or dermatologist will do a check-up to see if they can find out what is making you unwell. They will ask you about any symptoms you might be having such as an itch, pain and/ or burning sensation on the skin, skin conditions that may present similar to dermatitis, psoriasis, tinea or fungal skin infections, fever, night sweats, and weight loss.

Symptoms limited to skin may be the only signs of CTCL. You may feel anxious or concerned after the diagnosis is made, but these concerns often ease when you are reviewed by a specialist or specialist team.

Initial tests you may have:

Referrals

If your GP thinks you may have CTCL, they’ll refer you to a haematologist or dermatologist at a public hospital or in private practice for more tests. Haematologists are highly trained in conditions that affect blood and bone marrow. You may also be referred to a radiation oncologist (a specialist medical doctor specialising in the use of radiation therapy) if radiotherapy is included in your treatment plan.

You can bring a family member or friend with you to your appointments.

Timeframes

The timing of your specialist appointment will depend on your initial results of tests performed by your GP or doctor. For a small number of patients, an urgent appointment or hospital admission may be required, but for most people with no urgency, an appointment within four weeks is appropriate.

If you can’t get an appointment within these timeframes, follow up with your GP.

Travel to access tests, treatment and care

You may have to travel to see a specialist, undergo tests or access treatment, especially if you live in a rural or regional area. In some cases, you may have to leave home for an extended period. Accommodation and transportation support services are available, and it is encouraged to have family support with you whilst you undergo treatment.

See further information on Leukaemia Foundation’s accommodation and transport support by visiting their accommodation services page and transport services page.

Mental health and emotional well-being

A blood cancer diagnosis can affect your mental and emotional well-being. Many people being treated for a blood cancer experience a range of feelings and it is not uncommon to feel low, depressed, or anxious. Patient support organisations such as the Leukaemia Foundation or Cancer Council, your GP, or another health professional such as a psychologist or social worker can help you develop strategies to cope. They can help you access a mental health treatment plan if required.

Helpful tips

Let your doctor know what is most important to you in a specialist (e.g., that they specialise in ALL, cost, location, bedside manner, expertise).

Ask your GP to recommend trusted sources of information and support — you can share these with your family and friends too.

Talk to your GP/specialist about how to manage any other health conditions you may have during your blood cancer treatment and let them know if you have any concerns.

It’s a good idea to keep a written diary or digital record of treatment details and appointments with the GP and specialists. You can ask permission to record appointments. It’s also helpful to bring a list of questions and ask for a written treatment plan. This can be an important source of information for later in life.

Don’t ignore new signs and symptoms. Alert the GP or specialist. Trust yourself. It’s OKAY to be persistent.

The haematologist/specialist will do more tests to see if you have CTCL, to check how advanced it is, and to help determine the best treatment for you. It is important to note that staging for CTCL, especially in Sézary Syndrome, can fluctuate greatly overtime. The staging of CTCL does not directly link to that of other cancers and interpretation should rely on specialist advice. This process of working out if you have a medical problem is called making a diagnosis.

You might have one test or a mix of tests:

Your specialist may suggest other imaging tests depending on the type of lymphoma suspected.

These tests will give the specialist more information about the glioma, such as where exactly it is in your body, if the cancer is growing or has spread, and what stage it is at. This is called staging, which helps to work out the best treatment for you.

Timeframes

You should have most results back within eight weeks.

Your specialist will talk to you about your treatment options for CTCL. You may need more than one treatment type to get the best results, and you may require ongoing treatment to manage your CTCL for the rest of your life.

You might have one treatment or a mix of treatments:

Corticosteroids. Drugs that help change your immune response by reducing inflammation and are applied directly onto skin patches and plaques.

Light therapy. UV light is used to decrease inflammation in the skin.

Radiotherapy uses localised x-rays to kill cancer cells and stop the cancer growing.

Total skin electron therapy treats the skin surface and reduces patches and plaques caused by CTCL.

Extracorporeal photopheresis. A procedure in which blood is drawn from a vein and the white blood cells in the sample are treated with UV light (PUVA phototherapy), and then re-infused into the person’s vein. The UV light treatment helps to target the cancerous T cells and triggers the immune system to destroy the cells. This is most effective for specific types of more advanced CTCL.

Interferon. Drugs that stimulate the immune system to specifically suppress the CTCL cells. This is given as an injection under the skin.

Systemic chemotherapy. Drugs that are used to kill cancer cells to stop the cancer growing. Generally reserved for treatment in refractory or rapidly progressive and advanced CTCL. Chemotherapy for CTCL can be delivered intravenously (through IV) or orally in pill form. Some subtypes of CTCL are more likely to respond to chemotherapy than others.

Other more novel or directed treatments may be appropriate for you in discussion with your treating team. Clinical trials may also be considered during your treatment journey.

For more information, visit Cancer Treatment. Supportive care (treatment or services that support you through a cancer experience) are also available, often delivered through a multidisciplinary team that may include nurses, pharmacists, psychologists, physiotherapists and dietitians.

Timeframes

By evaluating your symptoms and test results, your specialist will determine when your treatment should start. For most people past early-stage disease, radiation therapy is rarely urgent and can be commenced within two to four weeks of the decision being made. There may be instances where your treatment could be delayed beyond this timeframe. If this occurs your specialist will discuss this with you.

You can ask your GP for a referral to another specialist for a second opinion.

For more information on clinical trials, visit the Australian Cancer Trials page or search the Australian New Zealand Clinical Trials Register. Participation in a clinical trial is voluntary, and you can choose not to participate in a clinical trial or withdraw from a clinical trial at any point if you wish.

For relationship support and, if necessary, referral to counselling services, visit the relationships and sex page on Leukaemia Austalia. Canteen Australia also provides some helpful information on fertility options after a cancer diagnosis.

You can ask for more time to think about your treatment options, and you can ask your GP for a referral to another specialist for a second opinion.

Lifelong treatment for your CTCL may be required, and this can affect your physical, psychological, nutritional, and general wellbeing. Due to the skin complications experienced with CTCL, such as patches, plaques, and broken skin, the risk for infection and sepsis is high and should be managed with your treating doctor.

Patients are urged to follow their prescribed skincare plan as intact skin provides protection from infection and sepsis. Comprehensive support and side effect management should be part of your routine ongoing care.

A number of specific needs may arise for patients at this time:

• general skin care including regular use of bland or non-perfumed moisturisers and/or topical steroids
• measures to reduce skin bacterial load such as bleach baths
• clothing that does not irritate the skin
• assistance to cope with the emotional distress and/ or anger of dealing with a potential cancer diagnosis, anxiety/depression, interpersonal problems, and adjustment difficulties
• management of physical symptoms including fatigue and higher likelihood of infection
• encouragement and support to increase levels of exercise

Ongoing care plan

Current therapies mean that most people with CTCL will go on to live long lives. You may need regular ongoing follow-up appointments and care, so your specialist and healthcare team will work with you to make a care plan for you and your GP. Your care plan may need to be updated over time if your needs change. This plan will explain:

• who your main contact person is after treatment
• how often you should have check-ups and what tests this will include
• understanding and dealing with potential side effects of treatment
• how to get help quickly if you have an urgent problem
• how to look after your overall health and wellbeing
• what healthcare and community support services are available to you and how to access them

Your specialist and healthcare team will talk to you about your needs and can refer you to other health professionals and community support services. Other information you get might be about:

• the side effects of treatment and the specialists you may need to see
• how to make healthy lifestyle choices to give you the best chance of staying well

For more information, visit After Cancer Treatment.

Most patients with CTCL will have a recurrence of their disease at some stage in their treatment journey, although the timeframe at which this happens is uncertain. While relapse or disease progression is common with CTCL, this does not always mean that the disease is behaving more aggressively or is likely to be life-limiting.

Access to the best available therapies, including clinical trials and treatment with a multidisciplinary team, are crucial to achieving the best outcomes for recurrence or progression of the disease.

Treatment will depend on the features presented in the recurrence of the disease, previous treatment, and the patient’s preferences.

In managing people with CTCL, treatment may include:

• biological therapies
• Immunomodulators
• monoclonal antibodies
• chemotherapy
• stem cell transplant
• participation in clinical trials

Advance care planning

Your GP or healthcare team may talk with you, your family, and carer about your future treatment and medical needs.

Advance care directive

Sometimes known as a living will, an advance care directive is a legally binding document that you prepare to let your family and healthcare team know about the treatment and care you might want or not want in case you become too unwell to make those decisions yourself.

For more information, visit Advance Care Planning Australia.

Palliative care

Your specialist may refer you to palliative care services, but this doesn’t always mean end-of-life care. Today, people can be referred to these services much earlier if they’re living with cancer or if their cancer returns. Palliative care can help you to live as well as you can, including managing pain and symptoms. This care may be at home, in a hospital or at another location you choose.

Speak to your GP or specialist or visit Palliative Care Australia.

Making treatment decisions

You may decide not to have treatment at all, or to only have some treatment to reduce pain and discomfort. You may want to discuss your decision with your healthcare team, GP, family and carer.

For more information, visit Advanced Cancer Treatment.

You may have to pay for some appointments, tests, medications, accommodation, travel or parking. Speak with your GP, specialist or private health insurer (if you have one) to understand what is covered and what your out-of-pocket costs may be.

If you have concerns about costs, talk to your healthcare team or a social worker about:

• being bulk-billed or being treated in the public system
• help with accommodation during treatment
• the possible financial impact of your treatment

You can call Cancer Council on 13 11 20 to speak to a healthcare professional about financial support. For more information about costs, visit Practical and financial assistance and What will I have to pay for treatment.