Skin lymphomas

What are skin lymphomas?

Skin lymphomas are specific types of non-Hodgkin lymphoma (NHL) where the disease manifests predominantly in the skin. The medical term used for skin is ‘cutaneous’. Most of these conditions are generally termed ‘primary cutaneous lymphomas’.

Cutaneous lymphomas are diseases that often require input from several professionals (e.g. pathologists, doctors, nurses) in order to reach a diagnosis and to manage the condition. The severity and management required is often based on the patient’s symptoms. Sometimes people with skin lymphoma will need extra assistance from psychologists and psychiatrists to help them manage emotionally.

Cutaneous T-cell lymphoma

What is cutaneous T-cell lymphoma (CTCL)?

CTCL is a general term for T-cell lymphomas that involve the skin including: mycosis fungoides and Sézary syndrome, the two most common types of CTCL.

Other types of CTCL include: lymphomatoid papulosis, peripheral T-cell lymphoma, cutaneous anaplastic large cell lymphoma, adult T-cell leukaemia/lymphoma, lymphomatoid granulomatosis, granulomatous slack skin disease, and pagetoid reticulosis, to name a few.

In addition to involving the skin, CTCL can involve the lymph nodes, blood and other internal organs

What are the symptoms of cutaneous T-cell lymphoma?

Mycosis fungoides:
This is the most common form of CTCL and it does not look the same for all patients. Mycosis fungoides syndrome is characterised with the following.

• Patch: Flat, red, scaly patches that may initially be mistaken for eczema, psoriasis or dermatitis.
• Plaques: Thick raised lesions that may look like a scab.
• Tumours: Raised bumps, which may or may not ulcerate (break down).
• Itching: A common characteristic.

Sézary syndrome:
This is another common form of CTCL, which distinguishes itself by the presence of lymphocytes in the blood. Sézary syndrome is characterised with the following:

• Erythroderma: Red, scaly, itchy skin. Patients with Sézary’s may have an entirely red body.
• Hyperkeratosis: Thickening of the skin on the palms and soles.
• Ectropion: Fragile nails, hair thinning, and thickening of the area around the eyelids.

Diagnosing skin lymphoma

Most people begin by going to see their GP with symptoms. If your doctor has concerns that you may have symptoms of a skin lymphoma they will refer you to a dermatologist or haematologist who may perform a biopsy. This means having a sample taken of the affected area that will be examined under a microscope to see if it contains cancer cells. If it does, you will need to have further tests.

These tests may include:

• physical examination
• blood tests
• chest x-rays
• bone marrow biopsy – some fluid is removed from the bone marrow and examined for cancer cells
• computed tomography (CT) scan – a specialised x-ray for building up three-dimensional pictures of the body
• positron Emission Tomography (PET) scan. A small amount of radioactive material is injected, which highlights cancerous areas when viewed with a special scanner.

How is cutaneous T-cell lymphoma treated?

Selecting a treatment for a patient depends on the symptoms, the patient’s general health and stage of disease.

A patient diagnosed with CTCL may undertake one or more of the following treatments as part of their treatment regimen.

Skin-directed therapies

• Topical chemotherapy – creams containing drugs which may be used to kill and prevent the growth and division of cancer cells
• Phototherapy UVB – broad and narrow band, and PUVA – the use of ultraviolet light to treat the skin
• Radiation therapy – the use of high energy x-rays to kill cancer cells and shrink tumours
• Topical steroids – creams containing man-made hormones

Systemic therapies:

• Photopheresis – a procedure in which a portion of a person’s blood is removed from the vein and the white blood cells in that portion of the blood are treated with PUVA phototherapy, and then re-infused into the person’s vein
• Retinoids – compounds chemically related to vitamin A
• Cytokines – naturally occurring compounds that are produced by human cells in response to triggers from the immune system
• HDAC inhibitors – enzymes that target DNA associated proteins (histones) and allow the cell’s genes to be expressed in a way that may help cancer cells die
• Chemotherapy – single drugs or a combination of drugs which may be used to kill and prevent the growth and division of cancer cells
• Stem cell autologous transplantation – the transplant of blood stem cells taken from self
• Stem cell allogeneic transplantation – the transplant of blood stem cells from one person to another. The donor is usually a sister or brother or an unrelated volunteer donor
• Clinical trials – test new treatments and compare them to established treatments, both to see if the new treatment works better, and to compare the side-effects

Cutaneous B-cell lymphoma

What is cutaneous B-cell lymphoma (CBCL)?

CBCL is a general term for B-cell lymphomas that involve the skin. Some B-cell lymphomas found on the skin may come from within the body but involve the skin. Therefore it is important when a skin biopsy shows a B-cell lymphoma that further tests are carried out to make sure other areas of the body are not involved and that the disease has originated in the skin.

Primary cutaneous follicle center lymphoma and primary cutaneous marginal zone B-cell lymphoma are the two most common types of CBCL. Other types of cutaneous B-cell lymphoma include primary cutaneous diffuse large B-cell lymphoma leg-type, primary cutaneous diffuse large B-cell lymphoma other, intravascular large B-cell lymphoma, T-cell rich large B-cell lymphoma, plasmablastic lymphoma and anaplastic B-cell lymphoma.

What are the symptoms of cutaneous B-cell lymphoma?

CBCLs may appear on the skin as a reddish rash, lump or nodule and may have a slightly raised and smooth appearance. Lesions can occur on any part of the body.

Primary cutaneous follicle center lymphoma
Has the following characteristics:

• may develop slowly over months or even years
• lesions may manifest as single or multiple tumours or nodules with a pink or reddish appearance
• lesions usually appear on the head, neck or trunk of the body.

Primary cutaneous marginal zone B-cell lymphoma
Has the following characteristics:

• may develop slowly over months or even years
• pink or red lesions, nodules and/or tumours
• lesions commonly found on the extremities.

How is cutaneous B-cell lymphoma treated?

• Radiotherapy (Radiation therapy) – the use of high energy x-rays to kill cancer cells and shrink tumours
• Surgery
• Topical steroids – creams containing man-made hormones
• Chemotherapy – single drugs or a combination of drugs which may be used to kill and prevent the growth and division of cancer cells
• Targeted therapy – single drug known as a monoclonal antibody or immunotherapy
• Stem cell autologous transplantation – the transplant of blood stem cells from self
• Stem cell allogeneic transplantation – the transplant of blood stem cells from one person to another. The donor is usually a sister or brother or an unrelated volunteer donor
• Clinical trials – test new treatments and compare them to established treatments both to see if the new treatment works better and to compare the side-effects. There are often new treatments being tested in clinical trials for CBCL. It is important to remember that today’s scientific research is continuously evolving. Treatment options may change as new treatments are discovered and current treatments are improved. Therefore, you may wish to check with your doctor for any treatment updates that may have recently emerged.