Overview of cutaneous (skin) lymphoma
Lymphoma is a type of cancer that starts in white blood cells called lymphocytes. These blood cells usually live in our lymphatic system, but are able to travel to any part of our body. They are important cells of our immune system, fighting infection and disease, and helping other immune cells work more effectively.
About Lymphocytes
We have different types of lymphocytes, with the main groups being B-cell lymphocytes and T-cell lymphocytes. Both B and T-cell lymphocytes have a specialised function, having an “immunological memory”. This means that when we have an infection, disease, or if some of our cells are damaged (or mutated), our lymphocytes examine these cells and create specialised “memory B or T-cells”.
These memory cells keep all the information about how to fight the infection, or repair the damaged cells if the same infection or damage happens again. This way they can destroy or repair cells much faster and more effectively next time.
- B-cell lymphocytes also make antibodies (immunoglobulins) to help fight infections.
- T-cells help regulate our immune responses so that our immune system works effectively to fight infections, but also helps to stop the immune response once the infection is gone.
Lymphocytes can become cancerous lymphoma cells
Cutaneous lymphomas happen when either the B-cells or T-cells that travel to your skin become cancerous. The cancerous lymphoma cells then divide and grow uncontrollably, or do not die when they should.
Both adults and children can get cutaneous lymphomas and most people with cutaneous lymphoma will have cancerous T-cells. Only about 5 out of every 20 people with cutaneous lymphoma will have a B-cell lymphoma.
Cutaneous lymphomas are also divided into:
- Indolent – Indolent lymphomas are slow growing and often go through stages where they are “sleeping” causing no harm to you. You may not need any treatment if you have an indolent cutaneous lymphoma, though some people will. Most indolent lymphomas do not spread to other parts of your body, though some can cover different areas of skin. Over time, some indolent lymphomas can become advanced in stage, meaning they spread to other parts of your body, but this is rare with most cutaneous lymphomas.
- Aggressive – Aggressive lymphomas are fast-growing lymphomas that can develop quickly and spread to other parts of your body. If you have an aggressive cutaneous lymphoma, you will need to start treatment soon after you are diagnosed with the disease.
Symptoms of Cutaneous Lymphoma
Indolent cutaneous lymphoma
You may not have any noticeable symptoms if you have an indolent lymphoma. Because indolent lymphomas are slow growing, they develop over many years, so a rash or lesion on your skin may go unnoticed. If you do get symptoms they may include:
- a rash that does not go away
- itchy or painful areas on your skin
- flat, reddish, scaly patches of skin
- sores that can crack and bleed and not heal as expected
- generalised redness over large areas of skin
- a single, or multiple lumps on your skin
- If you have a darker colour skin, you may have areas of skin that are lighter than others (rather than redness).
Patches, papules, plaques and tumours - What's the difference?
The lesions you have with cutaneous lymphomas can be a generalised rash, or may be referred to as patches, papules, plaques or tumours.
Patches – are usually flat areas of skin that are different to the skin around it. They can be smooth or scaly and can look like a general rash.
Papules – are small, solid raised areas of skin, and may look like a hard pimple.
Plaques – are hardened areas of skin that are often slightly raised, thicker areas of skin that are often scaly. Plaques can often be mistaken for eczema or psoriasis.
Tumours – are raised bumps, lumps or nodules that can sometimes become sores that do not heal.
Aggressive and Advanced cutaneous lymphoma
If you have an aggressive or advanced cutaneous lymphoma, you may have any of the above symptoms, but you may also have other symptoms. These can include:
- Swollen lymph nodes that you may be able to see or feel as a lump under your skin – these will usually be in your neck, armpit or groin.
- Fatigue which is extreme tiredness not improved with rest or sleep.
- Unusual bleeding or bruising.
- Infections that keep coming back or don’t go away.
- Shortness of breath.
- B-symptoms.
How is cutaneous lymphoma diagnosed?
You will need a biopsy or several biopsies to diagnose cutaneous lymphoma. The type of biopsy you have will depend on the type of rash or lesions you have, where they are located on your body and how big they are. It will also depend on whether it is only your skin that is affected, or if the lymphoma has spread to other parts of your body such as your lymph nodes, organs, blood or bone marrow. Some of the types of biopsies that may be recommended for you are listed below.
Skin biopsy
A skin biopsy is when a sample of your rash or lesion is removed and sent to pathology for testing. In some cases, if you have a single lesion, the entire lesion may be removed. There are different ways to perform the skin biopsy, and your doctor will be able to talk to you about the right skin biopsy for your circumstances.
Lymph node biopsy
If you have swollen lymph nodes that can be seen or felt, or that have shown up on scans, you may have a biopsy to see if the lymphoma has spread to your lymph nodes. There are two main types of lymph node biopsies used to diagnose lymphoma.
They include:
Core needle biopsy – where a needle is used to remove a sample of your affected lymph node. You will have a local anaesthetic to numb the area so you don’t feel pain during this procedure. In some cases, the doctor or radiologist may use an ultrasound to guide the needle into the right spot for the biopsy.
Excisional biopsy – with an excisional biopsy you will likely have a general anaesthetic so you sleep through the procedure. An entire lymph node or lesion is removed during and excisional biopsy so the entire node or lesion can be checked in pathology for signs of lymphoma. You will likely have a few stitches and a dressing when you wake up. Your nurse will be able to talk to you about how to care for the wound, and when/if you need to have the stitches removed.
Subtypes of indolent cutaneous T-cell lymphomas
Subtypes of indolent B-cell cutaneous lymphomas
Subtypes of Aggressive Lymphoma
Staging of Cutaneous Lymphoma
Once it has been confirmed that you have a cutaneous lymphoma, you will need to have more tests to see if the lymphoma has spread to other parts of your body.
Physical examination
Your doctor will do a physical examination and check the skin all over your body to see how much of your skin is affected by the lymphoma. They may ask for your consent to take photos so they have a record of what it looked like before you start any treatment. They will then use these to check if there is improvement with the treatment. Consent is your choice, you do not have to have photos if you do not feel comfortable with this, but if you do consent, you will need to sign a consent form.
Positron Emission Tomography (PET) Scan
A PET scan is a scan of your entire body. It is done in a special section of the hospital called “nuclear medicine” and you will be given an injection of a radioactive medicine that any lymphoma cells absorb. When the scan is taken, the areas with lymphoma then light up on the scan to show where the lymphoma and the size and shape of it.
Computed Tomography (CT) scan
A CT scan is a specialised X-ray that takes 3 dimensional pictures of the inside of your body. It usually takes a scan of an area of your body such as your chest, abdomen or pelvis. These images can show if you have swollen lymph nodes deep inside your body, or areas that look cancerous in your organs.
Bone Marrow Biopsy
Most people with cutaneous lymphoma will not need a bone marrow biopsy. However, if you have an aggressive subtype, you may need one to check if the lymphoma has spread to your bone marrow.
Two types of biopsies are taken during bone marrow biopsy:
- Bone marrow aspirate (BMA): this test takes a small amount of the liquid found in the bone marrow space
- Bone marrow aspirate trephine (BMAT): this test takes a small sample of the bone marrow tissue
TNM/B staging system for cutaneous lymphoma
The staging of cutaneous lymphoma uses a system called TNM. If you have MF or SS there will be an extra letter added – TNMB.
T = size of Tumour – or how much of your body is affected by the lymphoma.
N = lymph Nodes involved – checks if the lymphoma has gone to your lymph nodes, and how many lymph nodes have lymphoma in them.
M = Metastasis – checks if, and how far the lymphoma has spread inside your body.
B = Blood – (MF or SS only) checks how much lymphoma is in your blood and bone marrow.
TNM/B Staging of Cutaneous Lymphoma |
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Cutaneous Lymphoma |
Mycosis fungoides (MF) or Sezary Syndrome (SS) only |
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TTumouror skinaffected |
T1 – you only have one lesion.T2 – you have more than one skin lesion but the lesions are in one area, or two areas close together on your body.T3 – you have lesions across many areas of your body. |
T1 – less than 10% of your skin is affected.T2 – more than 10 % of your skin is affected.T3 – you have one or more tumours bigger than 1cm.T4 – you have erythema (redness) covering more than 80% of your body. |
NLymphNodes |
N0 – your lymph nodes appear normal.N1 – one group of lymph nodes are involved.N2 – two or more groups of lymph nodes are affected in your neck, above your clavicle, underarms, groin or knees.N3 – lymph nodes in, or near your chest, lungs and airways, major blood vessels (aortic) or hips are involved. |
N0 – Your lymph nodes appear normal.N1 – you have abnormal lymph nodes with low grade changes.N2 – You have abnormal lymph nodes with high grade changes.Nx – you have abnormal lymph nodes, but the grade is not known. |
MMetastasis(spread) |
M0 – none of your lymph nodes are affected.M1 – lymphoma has spread to your lymph nodes outside of your skin. |
M0 – none of your internal organs are involved, such as lungs, liver, kidneys, brain.M1 – lymphoma has spread to one or more of your internal organs. |
BBlood |
N/A |
B0 – less than 5% (5 out of every 100) cancerous lymphocytes in your blood.These cancerous cells in your blood are called Sezary cells.B1 – More than 5% of the lymphocytes in your blood are Sezary cells.B2 – More than 1000 Sezary cells in a very small amount (1 microliter) of your blood. |
Your doctor may use other letters such as “a” or “b” to further describe your lymphoma cells. These may refer to the size of your lymphoma, the way the cells look, and whether they have all come from one abnormal cell (clones) or more than one abnormal cell.Ask your doctor to explain your individual stage and grade, and what it means for your treatment. |
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Treatment for Indolent Cutaneous Lymphoma
Most indolent lymphomas cannot be cured yet despite this, many people with indolent cutaneous lymphomas will never need treatment.
Indolent cutaneous lymphomas are also usually not harmful to your health, so any treatment you have will be to manage your symptoms rather than cure your disease.
Some symptoms that may benefit from treatment include:
- pain
- itching
- wounds or sores that keep bleeding
- embarrassment or anxiety related to the way the lymphoma looks.
Types of treatment can include the following.
Local or skin directed treatment.
Topical treatments are creams or gels that you rub into the area of lymphoma, while skin directed therapy may include radiotherapy or phototherapy. Below is an overview of some treatments you may be offered.
Systemic treatments
If you have many areas of your body that are affected by the lymphoma, you may benefit from a systemic treatment such as chemotherapy, immunotherapy or targeted therapy. These are described in more detail under the next section – Treatment for aggressive cutaneous lymphoma.
Treatment for Aggressive or Advanced Cutaneous Lymphoma
Aggressive and/or advanced cutaneous lymphomas are treated similarly to other types of aggressive lymphoma and can include:
Systemic treatments
Stem cell transplant
Stem cell transplants are only used if your lymphoma does not respond to other treatments (is refractory), or comes back after a time of remission (relapse). It is a multi-step treatment where your own, or a donor’s stem cells (very immature blood cells) are removed through a procedure called apheresis, and then given to you at a later time, after you have had high dose chemotherapy.
With cutaneous lymphoma, it is more common for you to receive stem cells from a donor rather than your own. This type of stem cell transplant is called an Allogeneic Stem Cell transplant.
Extracorporeal photopheresis (ECP)
Extracorporeal photopheresis is a treatment used predominantly for advanced MF and SS. It is a process of “washing” your blood and making your immune cells more reactive to the lymphoma to cause the lymphoma cells to be killed. If you need this treatment, your doctor will be able to provide you with more information.
Clinical Trials
It is recommended that anytime you need to start treatment you ask your doctor about clinical trials you may be eligible for. Clinical trials are important to find new medicines, or combinations of medicines to improve treatment of cutaneous lymphoma in the future.
They can also offer you a chance to try a new medicine, combination of medicines or other treatments that you would not be able to get outside of the trial. If you are interested in participating in a clinical trial, ask your doctor what clinical trials you are eligible for.
There are many treatments and new treatment combinations that are currently being tested in clinical trials around the world for patients with both newly diagnosed and relapsed cutaneous lymphomas.
Treatment options for aggressive or late-stage cutaneous lymphoma | |
B-cell cutaneous | T-cell cutaneous |
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Also ask your haematologist or oncologist about any clinical trials you may be eligible for. | |
What happens when treatment doesn't work, or the lymphoma comes back
Sometimes treatment for lymphoma doesn’t work at first. When this happens it is called refractory lymphoma. In other cases, the treatment may work well, but after a time of remission the lymphoma may come back – this is called a relapse.
Whether you have a relapsed or refractory lymphoma, your doctor will want to try a different treatment that may work better for you. These next treatments are called second-line treatments, and may be more effective than the first treatment.
Talk to your doctor about what the expectations of your treatment are, and what will the plan be if any of them don’t work.
What to expect when treatment finishes
When you finish your treatment your specialist doctor will still want to see you regularly. You will have regular check-ups including blood tests and scans. How often you have these tests will depend on your individual circumstance and your haematologist will be able to tell you how often they want to see you.
It can be an exciting time or a stressful time when you finish treatment – sometimes both. There is no right or wrong way to feel. But it is important to talk about your feelings and what you need with your loved ones.
Support is available if you are having a difficult time coping with the end of treatment. Talk to your treating team – your haematologist or specialist cancer nurse as they may be able to refer you for counselling services within the hospital. Your local doctor (general practitioner – GP) can also help with this.
Lymphoma Care Nurses
You can also give one of our Lymphoma Care Nurses a call or email. Just click on the “Contact Us” button at the bottom of the screen for contact details.
Summary
- Cutaneous lymphoma is a subtype of Non-Hodgkin Lymphoma resulting from cancerous blood cells called lymphocytes, travelling to and living in the layers of your skin.
- Indolent Cutaneous Lymphomas may not need treatment as they are often not dangerous to your health, but you may have treatment to manage symptoms if they make you uncomfortable, or if the lymphoma spreads to your lymph nodes or other parts of your body.
- Aggressive Cutaneous Lymphomas need treatment soon after you are diagnosed.
- There are several different specialist doctors that may manage your care, and this will depend on your individual circumstances.
- If your lymphoma is affecting your mental health or mood you can ask your doctor for a referral to a psychologist to help you cope.
- Many treatments are aimed to improve your symptoms; however, you may also need treatments to manage the lymphoma, and these can include chemotherapy, monoclonal antibodies, targeted therapies and stem cell transplants.
