Sezary syndrome is a rare, chronic disease with many years of course, the cause of which has not been clearly established. What are the causes and symptoms of Sezary's syndrome? How is the treatment going?

Sezary's syndromescientists classify it in two ways - some believe that Sezary's syndrome is a variant of mycosis fungoides, some claim it is primary cutaneous lymphoma Type T is most often affected by older men, aged 60-70.

Sezary's syndrome: symptoms and diagnosis

Sezary's syndrome is characterized by erythrodemia, i.e. reddening of the skin of the entire body, intense itching, and lymphadenopathy. The skin becomes thickened, with exfoliation.

Typical for this unit is facial skin involvement, generalized hair loss (patients lose not only hair on the head, but also eyebrows, eyelashes, armpits and genital hair), and the appearance of nail changes.

The skin on the hands and feet is calloused, cracks forming deep gaps.

The peripheral blood of affected patients contains Sezary cells that resemble altered T cells.

Sezary syndrome: diagnosis

In order to diagnose Sezary's syndrome, it is necessary to perform a histological examination of a section of the affected skin and to confirm the presence of Sezary cells in the peripheral blood (detection of>1000 cells / mm 3 ).

Sezary's syndrome: treatment

The treatment of choice in Sezary's syndrome isextracorporeal photopheresis . It is a method of separating the lymphocytes from the patient's peripheral blood and irradiating them with UVA rays outside his body, after giving him drugs that are photosensitizers, i.e. they make the skin more sensitive to radiation.

Alternative methods of therapy are similar to the treatment of mycosis fungoides, are mainly based on light therapy: UVA and UVB ultraviolet irradiation, PUVA and RE-PUVA therapy.

In advanced cases of Sezary's syndrome, cytostatics are added to treatment.

Sezary's syndrome: prognosis

Sezary's prognosis is not good, average survival time is2-3 years.

Category:

Oncology