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Overview

Cutaneous T cell lymphoma (CTCL) is a lymphoma of T cell (a type of white blood cell that is responsible for immune function) origin that affects the skin. Its symptoms vary. Often it is confined to the skin and can be treated and often cured. Aggressive forms can extend beyond the skin to the lymph nodes, blood, and internal organs.

Cutaneous T cell lymphoma is frequently referred to as mycosis fungoides. In this chronic disorder, malignant T cells migrate to, and multiply in, the skin. Left untreated, these malignant cells may remain in the skin for years and sometimes for decades. They eventually spread to the lymph nodes, blood, or internal organs. Treatment, especially in early stages, can result in cure.

Causes

In normal skin reactions, T cells are recruited to the skin to fight off infectious invasion. The T cells divide and expand, and after the foreign stimulus is eliminated, they die off. In CTCL, the malignant T cells cycle and divide without dying off. Thus they remain in the skin and proliferate. The cause for this malignant proliferation is unknown, but certain viruses, like the human T cell lymphoma virus (HTLV), stimulate some variants of CTCL.

Signs and Symptoms

Clinically, as well as pathologically, CTCL has four stages of progression: patch stage, plaque stage, tumor stage, and erythroderma stage. The disease can manifest at any stage and does not necessarily go through an orderly progression. In addition, erythroderma (red skin) can develop, as well as a systemic blood disorder known as Sezary syndrome, which involves the migration of malignant T cells throughout the body.

The patch stage is characterized by flat, pink areas of skin, inflammation, and, sometimes, itching. The patches may remain for years and can resolve spontaneously. They can vary in size and are usually scaly. These nonspecific patches can make diagnosis difficult, clinically and pathologically, because they look like several other benign skin disorders.

In the plaque stage, the cells multiply and grow, and the lesions thicken, become elevated, and show textural changes. Plaques may be annular (forming a ring); pink, white, or brown; and are often scaly. As the plaques thicken, the diagnosis becomes more obvious.

In the tumor stage, multiple reddish brown or purplish red nodules develop. The face and body folds are usually the sites of tumor development. The tumor may ulcerate and become infected. Spontaneous resolution of tumors has been observed, but once tumor and ulceration occur, the disease is considered aggressive, and the prognosis is poor.

The erythroderma stage can arise in isolation or progress from any other stage. The skin is diffusely red and scaly. The palms and soles may become very thick and crack, making it difficult to walk. There are other causes of erythroderma that should be excluded, including psoriasis, eczema, or a medication allergy.

With Sezary syndrome, there is generalized redness, scaling, intense itching, and lymph node involvement, as well as malignant cells circulating in the blood.

Diagnosis

While diagnosis is often suggested by the appearance of the lesion, it is always confirmed with a biopsy (often multiple biopsies) and examination under the microscope. In the early stages, the diagnosis may be suspected for years before it is finally confirmed after repeated biopsies.

Any enlarged lymph nodes should be evaluated for malignant cells, as should the blood in cases of suspected Sezary syndrome. Extremely atypical cells imply a worse prognosis and the need for more aggressive therapy. A thorough examination should be undertaken to help guide treatment choices. The prognosis worsens as the disease spreads.

Treatment

The various presentations and extent of involvement affect prognosis and guide therapeutic decisions. In early stages of disease, more options are available and cures are achieved. Treatment is typically directed to the whole body, because there may be undetected lesions that should be treated. These lesions may temporarily become more apparent during therapy and then resolve.

Phototherapy may be useful in the treatment of CTCL, which involves exposure to controlled, progressive ultraviolet radiation. Either ultraviolet B (UVB) or UVA rays are used. An oral medication called Psoralen is used with UVA ray phototherapy, because the drug makes the skin more responsive to UVA rays. This combination therapy is also referred to as PUVA. Patients receiving UVB or PUVA are treated 3 times per week, until remission is achieved. At that point, treatments are tapered off, although monthly maintenance therapy may be continued for years. Phototherapy, particularly with PUVA, can increase one s risk for other types of skin cancer, and all patients should be checked periodically. Specialized glasses must be worn for 24 hours following each treatment, because Psoralen causes increased sensitivity to light.

Topical nitrogen mustard (a chemotherapeutic agent) can also be used for patch or plaque stage treatment. The dose of the medication, which the patient applies to his or her body, is gradually increased as tolerance develops. This therapy can induce long-term remission and even cures. Nitrogen mustard therapy can be used in combination with phototherapy to increase effectiveness.

Electron-beam therapy is a widely accepted treatment option. With electron-beam therapy, radiation affects the skin only and does not penetrate to the internal organs, such as the intestines or bone marrow. This greatly limits side effects.

Whole body electron-beam radiation results in complete remission in about 84% of individuals. Relapse of the cancer is seen more often in more advanced disease. This therapy is usually not a first choice, as the allowable lifetime amount of radiation is limited. Thus, the therapy is usually reserved for patients who do not respond to other forms of treatment.

Extracorporeal photochemotherapy (ECP) involves removing a portion of the patient's white blood cells from the body, treating them with UVA light, and returning them to the patient s body. These treatments are given on 2 consecutive days, once a month. This helps to treat and manage erythrodermic CTCL. While not all of the white blood cells respond to this treatment, even a partial response can make the disease more manageable and improve the patient's quality of life.

Chemotherapy is used to treat CTCL, but it is typically used only to alleviate pain. To date, no chemotherapeutic regimen has been curative in advanced cases of the disease.

Prevention
The etiology (cause) of CTCL is not well understood. In some cases, a virus has been responsible for development of this disease and exposure, when known, should be limited.

Skin Cancer, Cutaneous T Cell Lymphoma, Overview, Causes, Signs and Symptoms, Diagnosis, Treatment reprinted with permission from dermatologychannel.net
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