Overview of cutaneous T-cell lymphoma (CTCL)
Cutaneous (skin) T-cell lymphoma (CTCL) is a rare form of non-Hodgkin lymphoma (NHL).
The mutated T-cells migrate to the skin, where it often first presents as a rash. The rash can be itchy and hard to diagnose. Most skin lymphomas are indolent (slow growing) and stay localised (in the same area) to the skin.
Types of cutaneous T-cell lymphoma (CTCL) include:
- Mycosis fungoides is the most common subtype of CTCL, and accounts for approximately 50% of all cases of CTCL. This subtype can occasionally run in families, but this is very rare.
- Primary cutaneous anaplastic large-cell lymphoma is usually an indolent lymphoma. This subtype can affect people of all ages, including children but are more common in 45-60-age group.
- Lymphomatoid papulosis is a non-cancerous condition of the immune system. This is a precursor (pre-cancerous) for CTCL. This condition can occur at any age from childhood to middle age.
- T-cell skin lymphoma is more common in people who have had a transplant or in people who have HIV (human immunodeficiency virus).
Who is affected by cutaneous T-cell lymphoma (CTCL)?
Cutaneous T-cell lymphomas (CTCL) are more common in men than they are in women. CTCL are more common in older patients but also affect children. The average age of diagnosis is 55 years of age and genetic factors might be involved in the development of some types of T-cell skin lymphoma.
Symptoms of cutaneous T-cell lymphoma
Most cutaneous T-cell lymphomas begin as patches of dry, and discoloured skin (usually red). They are commonly located on the buttocks or between the waist and shoulders (torso). Yet, they can also develop in other places. These patches may be itchy and skin symptoms can also include:
- Patches of lighter or darker skin
- Patches of mottled skin
- Harder or thicker areas of skin called plaques
- Small, raised solid areas of skin called papules
- Larger swellings in the skin, called nodules or tumours, which can break down (ulcerate) and scab over
- Generalised reddening of the skin, which can be intensely itchy, dry and scaly
- Thickened or cracked skin on palms of hands or soles of the feet
Swollen lymph nodes in the neck, armpits or groin may also be present.
B symptoms may be present and can include:
- Night sweats (drenching sleepwear and bedding)
- Persistent fever
- Unexplained weight loss
- Itchy skin
It is important to tell the doctor about all symptoms as it can influence the decision around the type of treatment and when to start.
Diagnosis and staging of CTCL
Several skin biopsies may be required before a CTCL diagnosis is made. A biopsy is an operation to remove a lymph node or other abnormal tissue to look at it under the microscope by a pathologist to see what the cells look like. The biopsy can be done under local or general anaesthetic depending on what part of the body is being biopsied. The biopsy can be done one of three ways:
- Fine needle aspirate
- Core needle biopsy
- Excisional node biopsy
An excisional node biopsy is the best investigative option, as it collects the most adequate amount of tissue to be able to do the necessary testing for a diagnosis.
Diagnosis requires careful correlation of clinical and pathological findings by experienced practitioners. A range of practitioners may include dermatologists, haematologists, radiotherapy oncologists, pathologists and specialist nurse practitioners
Waiting for test results can be a difficult time. It may help to talk to your family, friends or a specialist cancer nurse.
Staging of CTCL
Once a diagnosis of CTCL is made, further tests are required to see where else in the body the lymphoma has affected or located. This is called staging.
Sezary syndrome and mycosis fungoides are staged in the same way. Stage 1 through to stage 4. The stages are determined by the amount of skin affected and whether there are abnormal lymphoma cells in the bloodstream. The staging is explained further in the website pages for mycosis fungoides and sezary syndrome.
All other T-cell skin lymphomas are staged using a system called TNM. TNM stands for:
Tumour, Nodes and Metastasis.
The staging of your lymphoma helps your doctor to know what’s the best treatment for you.
What is the ‘grade’ of Lymphoma?
Lymphomas are also often grouped as either indolent or aggressive. Indolent lymphomas are usually slow growing and aggressive lymphomas are fast growing. The grade is also referred to as the clinical behaviour of the lymphoma.
Staging scans and tests
The scans and tests needed for staging and before treatment can start may include:
- Positron emission tomography (PET) scan
- Computed tomography (CT) scan
- Bone marrow biopsy
- Lumbar puncture & magnetic resonance imaging (MRI) – If lymphoma is suspected in the brain or spinal cord
Patients may also undergo a number of baseline tests prior to any treatment commencing to check organ functions. These are often repeated during and after the treatment has completed to assess whether the treatment has affected the functioning of organs. Sometimes the treatment and follow-up care may need to be adjusted to help manage side effects. These may include:
- Physical examination
- Vital observations (blood pressure, temperature, & pulse rate)
- Heart scan
- Kidney scan
- Breathing tests
- Blood tests
It may take some time for all the necessary biopsies and tests to be done (an average of 1-3 weeks), but it is important for the doctors to have a complete picture of the lymphoma and the general health of the patient in order to make the best treatment decisions
Many of the staging and organ function tests are done again after treatment to check whether the lymphoma treatment has worked and the effect this has had on the body.
Prognosis of cutaneous T-cell lymphoma (CTCL)
Early stage CTCL can be managed well with the current therapies available today but most cases are considered incurable. However, CTCL can be treated as a chronic conditions and patients can live normal lives.
Treatment of cutaneous T-cell lymphoma (CTCL)
Once all the results from the biopsy and the staging scans have been completed, the doctor will review these to decide the best possible treatment for a patient. At some cancer centres, the doctor will also meet with a team of specialists to discuss the best treatment and this is called a multidisciplinary team (MDT)meeting.
Doctors take into consideration many factors about the lymphoma and the patient’s general health to decide when and what treatment is required.
This is based on:
- The stage of lymphoma
- Symptoms (including the size and location of the lymphoma)
- How the lymphoma is affecting the body
- Past medical history & general health
- Current physical and mental wellbeing
- Patient preferences
The standard first-line treatment for CTCL may include:
Early stage cutaneous T-cell lymphoma treatment
- Bexarotene (Targretin) gel and capsules
- Corticosteroids (topical and oral)
- Methotrexate (Methoblastin)
- PUVA- Psoralen Plus ultraviolet A (UVA) light
- UVB therapy
- You may have some treatments as part of a clinical trial
Advanced stage cutaneous T-cell lymphoma treatment
- Alemtuzumab (Campath)
- Bexarotene (Targretin) gel and capsules
- Brentuximab Vedotin (Adcetris)
- Carmustine (BCNU)
- CHOP chemotherapy (cyclophosphamide,doxorubicin, vincristine and prednisolone)
- Corticosteroids (topical and oral)
- Extracorporal Photopheresis
- Gemcitabine (Gemzar)
- Methotrexate (Methoblastin)
- Pralatrexate (Folotyne)
- Rituximab (Mabthera)
- Romidepsin (Istodax)
- Stem cell transplant (autologous or allogeneic)
- Vorinostat (Zolinza)
Psychological support is also important. Skin infections and skin pain often need managing.
Common side effects of treatment
There are many different side effects of the treatment and these are dependent on the treatment that has been given. The treating doctor and/or cancer nurse can explain the specific side effects prior to the treatment. Some of the more common side effects of treatment may include:
- Anaemia (low red blood cells carry oxygen around the body)
- Thrombocytopenia (low platelets that help bleeding and clotting)
- Neutropenia (low white blood cells help with immunity)
- Nausea and vomiting
- Bowel problems such as constipation or diarrhoea
- Fatigue (tiredness or lack of energy
The medical team, doctor, cancer nurse or pharmacist, should provide information about:
- What treatment will be given
- What are the common and possible side effects for the treatment
- What side effects do you need to report to the medical team
- What are the contact numbers, and where to attend in case of emergency 7 days a week and 24 hours per day
Once treatment has completed, post treatment staging scans are done to review how well the treatment has worked. The scans will show the doctor if there has been a:
- Complete response (CR or no signs of lymphoma remain) or a
- Partial response (PR or there is still lymphoma present, but it has reduced in size)
If all goes well regular follow-up appointments will be made for every 3-6 months to monitor the below:
- Review the effectiveness of the treatment
- Monitor any ongoing side effects from the treatment
- Monitor for any late effects from treatment over time
- Monitor signs of the lymphoma relapsing
These appointments are also important so that the patient can raise any concerns that they may need to discuss with the medical team. A physical examination and blood tests are also standard tests for these appointments. Apart from immediately after treatment to review how the treatment has worked, scans are not usually done unless there is a reason for them. For some patient’s appointments may become less frequent over time.
Relapsed or Refractory management of cutaneous T-cell lymphoma (CTCL)
As a principle chemotherapy is reserved for late stage CTCL and if patients relapse (cancer returns) , treatments can include:
- Antibody therapy with alemtuzumab and brentuximab vedotin.
- Stem cell transplant
Treatment under investigation for cutaneous T-cell lymphoma (CTCL)
There are many treatments that are currently being tested in clinical trials around the world and in Australia for patients with both newly diagnosed and relapsed lymphoma. New therapies for CTCL can include:
- Anti CD47 monoclonal antibodies
- Carfilzomib (Kyprolis)
- Lenalidomide (Revlimid)
- Mogamulizumab (Poteligio)
What happens after treatment?
Sometimes a side effect from treatment may continue or develop months or years after treatment has completed. This is called a late effect.
This can be a challenging time for many people and some of the common concerns can be related to:
- Mental wellbeing
- Emotional health
- Work, study, and social activities
Health and wellbeing
A healthy lifestyle, or some positive lifestyle changes after treatment can be a great help after the treatment has been finished. Making small changes such as eating and increasing fitness can improve health and wellbeing and help the body to recover. There are many self-care strategies that can help during the recovery phase.