Cutaneous T-cell lymphoma

The term cutaneous T-cell lymphoma (CTCL) refers to a skin condition in which there is an abnormal neoplastic proliferation of lymphocytes with a 'T' subtype (thymus-derived). The diagnosis is made by clinical appearance of your rash and skin biopsy.

Cutaneous T-cell lymphoma includes:

• Mycosis fungoides
• Sézary syndrome
• Anaplastic Large Cell Lymphoma
• T-cell leukemia
• Subcutaneous T-Cell Lymphoma

Mycosis fungoides is a condition in which the skin is infiltrated by patches or lumps composed of white cells called lymphocytes. It is more common in men than women and is very rare in children. Its cause is unknown.

Mycosis fungoides may persist in one stage or slowly progress to another stage.

• Patch stage: at this stage the skin condition is flat. Most often there are oval or ring-shaped pink dry patches on covered skin. They may spontaneously disappear, remain the same size, or slowly enlarge. The skin may be atrophic (thinned), and may or may not itch. The patch stage of mycosis fungoides can be difficult to distinguish from psoriasis, or eczema.
• Plaque stage: the patches become thickened and may resemble psoriasis. They are usually itchy.
• Tumour stage: large irregular lumps develop from plaques, or de novo. They may ulcerate. At this stage, spread to other organs is more likely than in earlier stages.

Sézary syndrome

The three typical features of Sezary Syndrome are: generalized red skin, enlarged lymph nodes, and atypical cells in the blood. The skin is also thickened, dry or scaly and usually very itchy. Examination of the blood usually reveals the presence of Sézary cells.

Anaplastic Large Cell Lymphoma

These lymphomas present as solitary or localized skin lesions, that have a tendency to ulcerate and may spontaneously regress.

Subcutaneous T-cell lymphoma

Typically, patients present with subcutaneous nodules, usually on legs, and weight loss, fever, and fatigue.

Investigations

The diagnosis of cutaneous T-cell lymphoma is made with the help of a dermatopathologist as there are characteristic microscopic changes seen on skin biopsy. The diagnosis is often delayed for months or years and may require several biopsies, as early cutaneous T-cell lymphoma can be difficult to tell apart from other skin conditions, particularly eczema.

Enlarged lymph nodes may also be biopsied. Cutaneous T-cell lymphoma can cause harmless swelling, known as 'reactive' or 'dermopathic' lymphadenopathy, or results in malignant growth in the lymph nodes.

The blood count is normal in most patients with cutaneous T-cell lymphoma, but presence of Sezary Cells is characteristic of Sézary syndrome.

Patients with advanced cutaneous T-cell lymphoma may have CT or MRI scans to determine whether the disease affects internal organs.

Treatment

Treatment of individual patients varies, and depends on the stage, local expertise and available drugs and equipment. The following may be useful:

• Topical steroids
• Topical nitrogen mustard
• PUVA Photochemotherapy
• UVB Phototherapy
• Localized radiotherapy
• Electron beam radiotherapy
• Chemotherapy
• Oral retinoids
• Interferons
• Photopheresis.

Prognosis

Cutaneous T-cell lymphoma often remains confined to the skin for many years, in some cases the abnormal cells may eventually infiltrate other tissues including blood, lymph nodes, lungs, heart, liver and spleen.

Unlike some other lymphomas, the outlook is generally good. Symptoms can usually be controlled with treatment. However treatment is not curative.

visit the Cutaneous T-cell Lymphoma site for more information