Cutaneous B-Cell Lymphoma (CBCL)

Primary Cutaneous Follicle Centre Lymphoma (PCFCL) is an indolent (slow growing) subtype of CBCL that usually affects people around the age of 60 years.

It usually develops on the back, abdomen, chest head or neck and rarely spreads anywhere else.

You may not need treatment for PCFCL because the lesions often remain stable for a long time, and some can even get better without treatment – though this rare.

If you need treatment for your PCFCL it may include radiotherapy or having the lesion surgically removed. You may also be given a corticosteroid cream to used. This usually results in a cure from the disease.

If your PCFCL is more widespread you may be offered a treatment with a monoclonal antibody called rituximab. These medications are given into your vein, and bind to the lymphoma cells. When they bind to the lymphoma cells they help your own immune system to find and destroy the lymphoma.

In some rare cases, PCFCL can transform into a faster growing subtype of lymphoma called Diffuse Large B-cell Lymphoma.

PC-MZL is an indolent subtype of CBCL and it is very rare for it spread past the skin. It is more common in people around 55 years of age.

There has been suggestion that some people of European descent that have had an infection with Borrelia Burgdorferi may have increased risk of developing this subtype of lymphoma. However, several studies have not been able to confirm this. Borrelia Burgdorferi is a bacteria related to the one that causes Lyme disease. If you do have this infection, you may be given antibiotics.

Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg type is a rare and aggressive subtype of CBCL. It is more common in women than men, and in people around 75 years of age. 

PC-DLBCL-LT often presents as fast-growing nodules, tumours or rash on one or both of your legs, but can spread to other parts of your body.  Some people with PC-DLBCL-LT can get B-symptoms. If you get B-symptoms, make sure to let your treating team know. B-symptoms are described further down this page but can include fever, weight loss and night sweats.

Although it is a cutaneous (skin) type of lymphoma, it is often treated with the same medications as other subtypes of Diffuse Large B-Cell Lymphoma, which includes chemotherapy and a monoclonal antibody. If you have only one lesion however, you may be treated with radiotherapy. You may go into remission after your treatment, but often this type of lymphoma comes back and needs more treatment at a later time. This is called a relapse.

Epstein-Barr virus (EBV) is the virus that causes glandular fever – also known as mononucleosis (mono), or the kissing disease, because it’s transmitted through saliva.

EBV+ Mucocutaneous  Ulcers are rare, but found in some people with compromised immune systems and a history of EBV infection. It is an indolent, and often self-limiting subtype of CBCL. Your risk of this rare subtype of CBCL is increased if you have had the Epstein-Barr virus and:

• have had a bone marrow, or organ transplant
• are on immune suppressive medications
• are older, and have a compromised immune system.

Most people with EBV+MCU will have one clearly defined ulcer. The ulcer may be found on the skin, gastrointestinal tract or in the mucosa of your mouth.

Treatment is often not needed, however, if you are on immune suppressing medications, your doctor may want to decrease your doses of these medications to allow your immune system to recover.

If this does not work, you may have treatment to to fight the Epstein-Barr virus, or an anti-cancer treatment called rituximab (a Monoclonal Antibody).