Skin lymphoma - cutaneous B cell lymphoma (CBCL)
Skin lymphoma is a rare type of non-Hodgkin lymphoma (NHL) that starts in the skin. NHL is a type of blood cancer that affects white blood cells called 
. It is a cancer of the .
Skin lymphoma is also called cutaneous lymphoma. There are 2 main types of skin lymphoma:
- cutaneous T cell lymphoma (CTCL)
- cutaneous B cell lymphoma (CBCL)
CTCL is the most common type of skin lymphoma. CBCL is more unusual. This page is about CBCL.
There are different types of CBCL including:
- primary cutaneous follicle centre lymphoma
- primary cutaneous marginal zone lymphoma
- primary cutaneous diffuse large B-cell lymphoma, leg-type
What is cutaneous B cell lymphoma?
Skin (cutaneous) lymphoma is a rare type of non-Hodgkin lymphoma (NHL).
Lymphoma develops when white blood cells called lymphocytes grow out of control. They move around your body in your blood and your lymphatic system.
The lymphatic system is an important part of our immune system. It has tubes that branch through all parts of the body.
These tubes are called lymph vessels or lymphatic vessels. They carry a straw coloured liquid called lymph. This liquid circulates around the body tissues. It contains a high number of white blood cells (lymphocytes) which fight infection.
There are 2 main types of lymphocytes:
- T cells
- B cells
In cutaneous lymphoma the T cells or B cells grow out of control within the skin. There are 2 main types of skin lymphoma:
- cutaneous T cell lymphoma (CTCL) starts in the T cells of the skin
- cutaneous B cell lymphoma (CBCL) starts in the B cells of the skin
CTCL is the most common type of skin lymphoma. It causes flat red patches on the skin that look like eczema and can be itchy. Several parts of the body can be affected.
CBCL is a more unusual type. People tend to have lumps on their skin in 1 or 2 areas, rather than affecting all of the body.
Symptoms of CBCL
Skin lymphoma can resemble other skin conditions such as eczema and psoriasis. So it can take a while for your GP to make a diagnosis and refer you to a specialist.
Small raised lumps (papules)
The main symptom of cutaneous B cell lymphoma is a small raised lump or lumps in the skin. They can look like spots or pimples. Doctors call these papules. You are most likely to have these on the head, neck, back or legs.
The papules can change to become thicker and flattish in their appearance. These are called plaques.
Larger lumps (nodules)
You might have larger lumps which are called nodules or tumours. These might be dark red or purple in colour.
You might have several lumps grouped together or you may have larger areas that are affected. It is possible for the lumps to break down (ulcerate).
Swollen lymph nodes
You might have swollen in your neck, armpit or groin.
Other symptoms
It’s rare to have symptoms such as weight loss, fever or night sweats.
Getting diagnosed
You might see different types of doctors during your diagnosis and treatment. There will be a team of people (multidisciplinary team ) from different hospital departments involved in your diagnosis. This might include:
- a dermatologist (specialist skin doctor)
- a haematologist (a specialist doctor who treats diseases affecting the blood)
- an oncologist (a cancer specialist)
Doctors diagnose CBCL by taking a sample of the affected skin. This is called a biopsy. A specialist doctor (pathologist) examines it under a microscope, looking for cancerous B cells.
Diagnosing skin lymphoma can be difficult. So it is not unusual to have more than one biopsy over a number of weeks or months.
If your doctor diagnoses lymphoma, you might have further tests. These might include:
- blood tests
- a
CT scan or PET-CT scan - a
lymph node biopsy if you have swollen lymph nodes - a
bone marrow test
Types and grades of cutaneous B cell lymphoma (CBCL)
Grade
Grade describes how the cells look under a microscope. Your grade tells the doctor how quickly the lymphoma is likely to grow and spread. Doctors put NHL into 2 grades:
- low grade (slow growing)
- high grade (fast growing)
There are different types of CBCL. Most are slow growing (low grade) but some can be fast growing (high grade).
Knowing the type and grade of the lymphoma helps doctors decide on the best treatment and how soon it should start. For some very slow growing types of CBCL, treatment might not need to start straight away.
Types of low grade CBCL
The term primary means the lymphoma has started in the skin, rather than spreading to the skin.
Primary cutaneous follicle centre lymphoma (PCFCL)
PCFCL is a low grade CBCL. It is the most common type. Around 60 out of 100 (60%) CBCL are this type. It tends to affect men more than women. It usually affects people around the age of 50.
PCFCL usually appears as firm, smooth nodules or plaques on the skin. These might be pink, red or purple. It often affects the head, neck, chest or back. You might have a single nodule or plaque. Or you may have several nodules that are grouped together.
PCFCL responds very well to treatment but it can come back (recur). It is very rare for it to change (transform) to a high grade lymphoma.
Primary cutaneous marginal zone lymphoma (PcMZL)
PcMZL is also called extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma).
It is most common for MALT lymphomas to develop in the gastrointestinal tract. The skin is the second most common site for MALT type lymphomas.
PcMZL is a low grade lymphoma. It is more common in men than women. It usually affects people around the age of 50. But it can occur in younger and older people too.
It usually appears as lumps, plaques or nodules. These might be red or pink in colour. They might develop on your chest, back, arms and sometimes your legs. They are slow growing and may be in one place or several places on your body.
In Europe, some cases of pcMZL are thought to be linked to the bacteria Borrelia burgdorferi. This is a bacterium carried by ticks that causes Lyme disease. But most people with Lyme disease do not develop lymphoma of the skin. You doctor might do tests to see if you have been exposed to Lyme disease. Treating Lyme can clear the lymphoma.
PcMZL does not usually spread and responds well to treatment. It can come back but responds well to further treatment.
Rarely pcMZL can transform into a high grade lymphoma
Types of high grade CBCL
Primary cutaneous diffuse large B cell lymphoma, leg type
Primary cutaneous diffuse large B cell lymphoma, leg type is a rare high grade lymphoma. Around 4 out of 100 (around 4%) of all skin lymphomas are this type. It generally affects people over the age of 70 and is more common in women.
It usually develops as large red-bluish nodules or tumours. These grow on the lower legs. They can develop in other places but this is less common. They grow quickly over a few weeks or months. The nodules and tumours can break down (ulcerate).
Stages of CBCL
The stage shows how much of the skin is affected by lymphoma, and whether it has spread to the lymph nodes or other body organs. Knowing the stage helps your doctor decide on the right treatment for you.
Doctors use the TNM system to stage CBCL.
The TNM staging system stands for Tumour, Node, Metastasis.
- T describes how many papules, nodules or tumours there are and how big they are
- N describes whether there are any cancer cells in the lymph nodes
- M describes whether the lymphoma has spread to a different part of the body
For CBCL, only the T category is used at the time of diagnosis. If at diagnosis other parts of the body (such as the lymph nodes) are affected by the lymphoma, the disease is not classed as skin lymphoma and is staged like non-Hodgkin lymphoma.
The N and M categories are only used if the lymphoma continues to grow during treatment or comes back after treatment.
T stages
There are 3 main T stages for CBCL - T1 to T3.
T1 means there is only one area (lesion) of lymphoma on the skin. It is split into 2 groups:
- T1a - a single lesion smaller than 5cm
- T1b - a single lesion 5cm or more
T2 means there are two or more lesions on the skin. This might be in one area of the body or on two areas of the body that are next to each other. T2 is divided into 3 groups:
- T2a - all of the skin lesions fit into a circular area measuring less than 15cm
- T2b - the skin lesions fit into a circular area between 15 and 30cm
- T2c - the skin lesions fit a circular area of 30cm or more
T3 means there are lesions in several areas of the body (generalised skin involvement).
N stages
There are 4 N stages:
N0 - there are no lymphoma cells in the lymph nodes
N1 - there are lymphoma cells in the lymph nodes that drain the area around the skin lymphoma
N2 – there are lymphoma cells in 2 or more sets of lymph nodes OR there are lymphoma cells in lymph nodes that do not drain from the area where the skin lymphoma is
N3 - there are lymphoma cells in the lymph nodes in the chest or tummy (abdomen)
M stages
There are 2 M stages:
M0 - the lymphoma has not spread away from the skin or lymph nodes
M1 - the lymphoma has spread to other parts of the body
Treatment for CBCL
The aim of treatment for CBCL might be to cure single, early stage lymphoma. For larger or more widespread lymphoma, the treatment might aim to control the lymphoma for as long as possible. And to control any symptoms you have.
Talk to your doctor or specialist nurse if you want to find out more about the aim of your treatment.
Your treatment depends on:
- where your lymphoma is (the stage)
- your symptoms
- your general health
The main treatments
The main treatment options for CBCL are:
- surgery
radiotherapy chemotherapy targeted immunotherapy drug
You might not have any treatment for your CBCL.
Treatment for low grade CBCL
This includes primary cutaneous follicle centre lymphoma and primary cutaneous marginal zone lymphoma.
Your doctor will give you antibiotics if you have primary cutaneous marginal zone lymphoma and your blood tests show that you have the Borrelia burgdorferi bacteria. You might not need further treatment.
Early stages
You might have surgery to remove a small area of lymphoma on the skin.
You might have radiotherapy after surgery. Or you can have radiotherapy as your main treatment instead of surgery if you have a few areas of lymphoma.
More advanced stages
You might not have treatment straight away if you don’t have any symptoms that are bothering you. This means you won’t have to experience any unpleasant side effects of treatment. Your doctor keeps a close eye on you. This is called active monitoring, or watch and wait. You start treatment when you need it.
If you have symptoms, you might have:
- a chemotherapy drug called chlorambucil
- a targeted cancer drug called rituximab
- a combination of chemotherapy and rituximab (chemoimmunotherapy) - for example R-CVP or R-bendamustine
Your specialist will decide which is the best treatment for your situation.
Treatment for high grade B cell skin lymphoma
This includes primary cutaneous diffuse large B cell lymphoma, leg type.
You usually have chemotherapy and a targeted cancer drug called rituximab. A common combination of drugs is called R-CHOP.
You usually have radiotherapy after chemotherapy to reduce the chance of the lymphoma coming back.
You may have radiotherapy on its own if you are not well enough for chemotherapy.
Survival for CBCL
The best person to talk to you about your outlook or prognosis is your doctor or specialist nurse. Not everyone wants to know. People cope differently with their lymphoma and want different information.
Survival depends on many factors. So no one can tell you exactly how long you will live. Your doctor might be able to give you some guide, based on their knowledge and experience.
Coping for CBCL
It can be difficult coping with a rare type of cancer as you may not meet many people in a similar situation. It can also be hard to live with the symptoms, such as itchy, sore and dry skin. Other people might notice your skin and it could make you conscious of your condition.
Ask your consultant or specialist nurse for advice about how to look after your skin and ways to make it more comfortable.
You could also ask if there are any support groups at your hospital or within your local area. There are also charities, such as the Lymphoma Association who can provide support and information.
It might help to let friends and family know if you are finding it difficult to cope. Talking about how you feel can help. Some people decide to have counselling.
