This disease affect its the name of a lymphoma. This disease affect its the name of which is an essential part of the immune system, which are responsible for immunity from bacteria and other microorganisms. from Albert Sezary, who first mentioned 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 most common form of cutaneous T-cell lymphoma, cutaneous, and means "incorporating the skin." Most of the patients diagnosed with the disease are between 55 and 60 years of age. In the western areas, this disease occur 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
Several have theorized that this is the late stage of mycosis fungoides, with lymphadenopathy (lymph node enlargement).
Signs and Symptoms
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, generally bathing trunk distribution (erythroderma), and;
3. Plaques / patches are with heterogeneity in presentation (the plaques / patches are different from each other.)
However 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 These approach 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 & # 39; 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 in 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 proved very effective in comparison to others, for the treatment for all case remaining difficult.
