Cutaneous T-cell lymphoma: symptoms, treatment and outlook

Lymphoma Action says most T-cell skin lymphomas begin as patches of dry, discoloured skin, usually red, commonly on the buttocks or between the waist and shoulders, but they can develop elsewhere. They develop slowly, often over years, and may be itchy or sometimes painful. BAD says the most common symptom of mycosis fungoides is long-term, generalised itching.

Both sources stress that early patches can look like common skin conditions such as eczema, dermatitis or psoriasis. This is why diagnosis can take a long time. Lymphoma Action reminds readers that T-cell skin lymphomas are rare and many conditions look similar, so these skin changes are much more likely to be something else.

• Patch stage: irregular, oval or ring-shaped, dry or scaly flat patches, often on skin not usually exposed to the sun.

• Plaque stage: patches that thicken into raised areas, which can be itchy and sometimes break down (ulcerate).

• Tumour stage: larger raised lumps that can ulcerate, weep and be painful; only a small proportion of people develop them.

• Erythroderma: skin that becomes red, thickened and sore all over, which is uncommon.

• Other possible features: lighter or darker patches, thickened or cracked skin on the palms or soles, hair loss in affected areas, and swollen lymph glands in the neck, armpits or groin.

• A small number of people have weight loss, fevers or night sweats, and Lymphoma Action advises seeing a GP if you are concerned about these.

BAD says the aim of treatment is to control symptoms and improve quality of life, and that no treatments have been shown to improve survival, although with early diagnosis and treatment people often live for many years without symptoms. Periods with no treatment are common. Lymphoma Action says mycosis fungoides can be very well controlled with treatment but tends to come back when treatment is stopped, and a few people do not need treatment at first.

The first step is good skin care with regular moisturisers (emollients). Most people only ever need skin-directed therapy, which treats the skin alone. If that does not control the condition, the case is discussed by a specialist skin lymphoma multidisciplinary team and other treatments may be offered; BAD says most patients do not need these.

Sézary syndrome affects the blood as well as the skin and needs whole-body treatment. Lymphoma Action says the first choice is usually extracorporeal photopheresis (ECP), which may be combined with methotrexate, bexarotene or interferon-alfa.

Lymphoma is a cancer that starts in white blood cells called lymphocytes. Lymphoma Action explains there are two kinds, T cells and B cells, and that a skin lymphoma starts in the skin; a lymphoma that starts elsewhere and spreads to the skin is not a skin lymphoma. T-cell skin lymphoma, also called cutaneous T-cell lymphoma (CTCL), is the commonest group. BAD says that in CTCL the T-cells develop abnormalities that make them attack the skin.

Lymphoma Action says skin lymphoma is a rare and complex condition that can be hard to diagnose, that in most cases it does not affect life expectancy, and that it is treated more like a chronic condition.

Lymphoma Action describes many types, most of them very rare, and classes them as low-grade (slow-growing) or high-grade (fast-growing). It says most skin lymphomas are low-grade, and that sometimes a slow-growing type changes into a faster-growing type, which is rare.

BAD says skin biopsies, taken under local anaesthetic, are usually needed to confirm the diagnosis. Because the rash and biopsies may not show specific features, biopsies may have to be repeated several times over a period, and blood test results are usually normal. A firm diagnosis may take months or even years. Lymphoma Action says the time taken does not usually affect the prognosis.

Staging describes how far the lymphoma has spread. Lymphoma Action says tests may include a physical examination, blood tests, a blood test to measure circulating lymphoma cells, a CT or PET/CT scan, and sometimes a lymph node biopsy. Mycosis fungoides and Sézary syndrome share a four-stage system.

BAD says the cause of mycosis fungoides is unknown, you cannot catch or pass it on, and it is not hereditary, although there have been very rare cases in which several family members developed it. Lymphoma Action says scientists do not know exactly what causes skin lymphoma, but some factors might increase risk.

These include age (two thirds of people are over 70), sex (men are around one and a half times more likely than women), possibly genetics, a virus called HTLV-1 for one subtype (adult T-cell leukaemia/lymphoma), and being a transplant recipient or having HIV. Most people with HTLV-1 do not develop lymphoma.

Lymphoma Action says that in most cases skin lymphomas are long-term conditions that are not life-threatening, and treatment usually aims to control symptoms rather than cure. Most low-grade skin lymphomas never develop beyond early stages and problems come and go. With treatment, many people with advanced disease have long periods free of symptoms, although the lymphoma may come back more quickly than earlier-stage disease.

BAD says mycosis fungoides is a life-long condition that usually progresses slowly over many years, that it is difficult to predict how it will affect each person, and that many people live a normal life span. Some skin lymphomas are faster-growing and need stronger treatment urgently, which is why a specialist should confirm the exact type.

Follow-up depends on the type: Lymphoma Action says you might see your specialist every 6 to 12 months, or move to patient-initiated follow-up, if your lymphoma is slow-growing and stable, and as often as every 3 months if it is fast-growing.

See a GP if you have a rash that does not respond to treatment for eczema or psoriasis, patches that slowly spread or thicken over months, long-term widespread itching, or lumps in the skin. Ask for the skin to be reassessed or biopsied if a rash keeps returning, because BAD says early patches may look like common conditions and biopsies often need repeating.

Go to your GP promptly for unexplained weight loss, fevers, night sweats or swollen glands. If you have been diagnosed and develop a rapidly growing or bleeding skin lump, a spreading red painful rash, or a high temperature, contact your skin lymphoma team or NHS 111, as infection is more likely when skin is broken or the immune system is affected.

On the NHS the route is usually GP to dermatology, then to a designated skin lymphoma clinic. BAD says difficult cases and those needing more than skin-directed therapy are discussed by a Supranetwork Skin Lymphoma Multidisciplinary Team, and there are several of these teams across the UK. Lymphoma Action says you might be referred to a specialist centre for treatment.

Private dermatologists can carry out biopsies and start skin-directed treatment, but advanced treatments and multidisciplinary discussion run through the specialist services. Ask for copies of your biopsy reports and make sure your GP and specialist team share the same information.