Overview of cutaneous B-cell lymphoma (CBCL)?
Cutaneous B-cell lymphomas (CBCL) are non-Hodgkin lymphomas (NHL) that start in the skin. CBCL are skin lymphomas that develop from B-cells. Some are indolent (slow growing) and some are aggressive (fast-growing).
Types of cutaneous B-cell lymphoma (CBCL)
Cutaneous B-cell lymphoma (CBCL) can affect people of all ages and there are three different types of CBCL that include:
- Primary cutaneous follicle centre lymphoma is an indolent (slow-growing) B-cell lymphoma. It is common in the western world and affects older patients, with the average at diagnosis is 60 years.
- Primary cutaneous marginal zone lymphoma is an indolent (slow-growing) B-cell lymphoma. It affects men twice as much as women. Some cases are associated with Lyme disease in endemic areas of Europe and the USA. Younger patients and children can be affected, however the average age at diagnosis is 55 years.
- Primary cutaneous diffuse large B-cell lymphoma, leg type is an aggressive (fast-growing) B-cell lymphoma. This subtype is twice as common in women than in men. It affects older people, with the average age at diagnosis of 75 years.
Symptoms of cutaneous B-cell lymphoma (CBCL)
The main symptom of cutaneous B-Cell lymphoma (CBCL) is a lump or lumps on the skin. The lumps often appear as small, raised, solid areas of skin that look like small pimples. These are called papules and may develop into thickened, flat areas or larger lumps can be deep red or purplish and are called nodules or tumours. Sometimes these lumps break down and ulcerate and may become infected.
Other symptoms may include:
- Swollen lymph nodes in your neck, armpits, and groin
B symptoms and these can include:
- Night sweats (drenching sleepwear & bedding)
- Persistent fevers
- Unexplained weight loss
Diagnosis of cutaneous B-cell lymphoma (CBCL)
A skin biopsy is always required for a diagnosis of lymphoma. A biopsy is a procedure to remove abnormal tissue to look at it under the microscope by a pathologist (a specialist doctor). The biopsy can be done under local or general anaesthetic depending on what part of the body is being biopsied.
B-cell lymphoma can be difficult to diagnose and several skin biopsies may be needed before the doctor is able to confirm the diagnosis. A specialist lymphoma centre may need to assist with diagnosis of these rare lymphoma subtypes.
Waiting for results can be a difficult time and it may help to talk to family, friends, or a specialist nurse.
Staging of cutaneous B-cell lymphoma (CBCL)
Once a diagnosis of CBCL is made, further tests are required to see where else in the body the lymphoma has spread and how much of the body is affected. This is called staging.
There are four stages from stage 1 (lymphoma in one area) through to stage 4 (lymphoma that is widespread).
- Early stage means stage 1 and some stage 2 lymphoma. This may also be referred to as ‘localised’. Stage 1 or 2 means that the lymphoma is found in one area or a few areas close together.
- Advanced stage means the lymphoma is stage 3 and stage 4, and is widespread In most cases, the lymphoma has spread to parts of the body that are far from each other.
B-cell skin lymphomas are staged using a system called ‘TNM’. TNM stands for Tumour, Nodes and Metastasis.
Tumour is how many and where they are. Nodes are how many are involved. Metastasis is whether the lymphoma has spread to any other part of the body.
What is the ‘grade’ of CBCL
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 B-cell lymphoma (CBCL)
Cutaneous follicle centre lymphoma (slow-growing) and cutaneous marginal zone B-cell lymphoma (slow-growing) both have a good prognosis. They are often diagnosed early, grow slowly, and respond well to treatment.
Cutaneous diffuse large B-cell lymphoma, leg type behaves as an aggressive (fast-growing) lymphoma They do respond to treatment but are highly likely to relapse (come back).
Treatment of cutaneous B-cell lymphoma (CBCL)
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
Cutaneous marginal zone lymphoma may be treated with antibiotics if the patient is infected with Borrelia burgdorferi.
The standard first-line treatment for indolent (slow-growing) cutaneous B-cell lymphomas can include:
- Surgery to remove the lesion followed by Radiotherapy
- Radiotherapy on its own if there are a few lesions
- Topical corticosteroids
- Topical chemotherapy such as Nitrogen mustard and carmustine
- Immune response modifiers such as Imiquimod
- Retinoids such as Bexarotene
- PUVA phototherapy
- ‘Watch and wait’ if there are no troublesome symptoms
- Rituximab and Bendamustine
- R-CVP (Rituximab, cyclophosphamide, vincristine, and prednisolone)
Standard first-line treatment for aggressive cutaneous B-cell lymphomas may include:
- Chemotherapy followed by Radiotherapy
- CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone)
- R-CHOP (Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone)
- Stem cell transplantation -allogeneic
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 CBCL
If Indolent cutaneous B-cell lymphoma relapses (comes back) after treatment, it can still be successfully treated with another treatment.
Cutaneous B-cell lymphoma leg type can be treated with different combination chemotherapies.
Treatments under investigation for CBCL
There are many treatments that are currently being tested in clinical trials in Australia and around the world for patients with both newly diagnosed and relapsed lymphoma. Some of the treatments for CBCL can include:
- Panobinostat (Farydak)
- Lenalidomide (Revlimid)
- Bendamustine (Treanda)
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.