Sézary syndrome

Sézary syndrome is a type of cutaneous T-cell lymphoma, a group of disorders that occur when T-cells (a type of white blood cell) become malignant and affect the skin. It is named after the French dermatologist Albert Sézary, who first described the condition in 1938.

Symptoms

The most common symptoms of Sézary syndrome include red, itchy skin (pruritus) that covers a large portion of the body and changes in the appearance of the nails, hair, and eyelids. Other symptoms may include enlarged lymph nodes, abnormal red blood cells (erythroderma), and a high white blood cell count.

Causes

The exact cause of Sézary syndrome is unknown. However, it is believed to be a result of a mutation in the T-cells. This mutation causes the cells to grow and divide uncontrollably, leading to the symptoms of the disease.

Diagnosis

Diagnosis of Sézary syndrome is often challenging due to its rarity and the similarity of its symptoms to other skin conditions. It is typically diagnosed through a combination of skin biopsies, blood tests, and imaging tests to check for enlarged lymph nodes.

Treatment

Treatment for Sézary syndrome often involves a combination of skin-directed therapies (such as topical corticosteroids and phototherapy) and systemic therapies (such as chemotherapy and biological therapy). The choice of treatment depends on the severity of the disease and the patient's overall health.

Prognosis

The prognosis for Sézary syndrome varies widely and depends on several factors, including the stage of the disease at diagnosis, the patient's age and overall health, and the response to treatment.

See also

• Cutaneous T-cell lymphoma
• Lymphoma
• T-cell