Categorized | Skin Care

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Sezary Syndrome

Sezary disease is an uncommon malignancy, and is a rare type of a lymphoma. This disease affects the T-cells, which are an essential part of the immune system, which are responsible for immunity from bacteria and other microorganisms. The disease derived its name from Albert Sezary, who first described the disease. It is known to be the late stage of mycosis fungoides, not a fungal infection, but also a type of malignancy.

Occurrence

Mycosis fungoides is the one of the most common form of cutaneous T-cell lymphoma, cutaneous, and means “involving the skin”. Most of the patients diagnosed with the disease are between 55 and 60 years of age. In the western areas, this disease occurs on 0.3 patients per 100,000 populations. This syndrome is more common in males than in females, with a ratio of 2:1.

Causes

The causes of having this type of carcinoma are unknown. Several have theorized that this is the late stage of mycosis fungoides, with lymphadenopathy (lymph node enlargement).

Signs and Symptoms

The disease is commonly manifested in the skin. The following can be observed in a patient with this disease:

1. Generalized erythroderma, which is characterized by skin redness, which is accompanied by warmth on the surrounding area. This is caused by dilation of the capillaries under the lesion;

2. Indolent cutaneous eruptions with erythematous scaly patches or plaques, typically bathing trunk distribution (erythroderma), and;

3. Plaques/patches are with heterogeneity in presentation (the plaques/patches are different from each other.)

Although the disease can present itself on the skin, it can also manifest with some symptoms in different systems of the body, which may present as:

Lymphadenopathy (lymph node enlargement);

Atypical T-cells (“Sezary cells”) in the peripheral blood (which is verified through pathology);

Hepatosplenomegaly (enlargement of the liver and the spleen).

Diagnosis

Diagnosis of Sezary disease will be done according to the doctor’s approach. These methods vary from:

1. Symptom analysis: the patient will be examined, and will present lesions that do not heal, even though medications are already prescribed;

2. Laboratory tests: an ordinary Complete Blood Count (CBC) can be ordered, and an increase in white blood cells (lymphocytes) can be noted. This increase is generally associated with cutaneous T-cell lymphoma;

3. Pathology: a smear from the patient’s skin lesions can show Sezary cells, which is a characteristic of the disease.

Treatment

A drug called vorinostat (brand name, Zolinza) is one of the drugs used for the treatment of cutaneous T-cell lymphoma. Treatment and management of the disease include a combination with phototherapy (treatment using light) and chemotherapy (using chemical agents/drugs). No single treatment type has been proven very effective in comparison to others, for the treatment for all cases remains difficult.

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