Sézary Syndrome
Sézary Syndrome is a rare, aggressive form of cutaneous T-cell lymphoma characterized by the widespread redness and scaling of the skin (erythroderma) and the presence of malignant T-cells (Sézary cells) in the blood. It is named after the French dermatologist Albert Sézary, who first described the condition in 1938.
Symptoms[edit | edit source]
The primary symptom of Sézary Syndrome is erythroderma, which covers most, if not all, of the body. Other symptoms may include intense itching (pruritus), enlarged lymph nodes (lymphadenopathy), and abnormalities of the fingernails and toenails. Some patients may also experience hair loss (alopecia) and a thickening of the palms of the hands and soles of the feet (palmoplantar keratoderma).
Causes[edit | edit source]
The exact cause of Sézary Syndrome is unknown. However, it is known to involve a mutation in the T-cells, a type of white blood cell that plays a crucial role in the body's immune system. This mutation causes the T-cells to become malignant and proliferate uncontrollably, leading to the symptoms of the disease.
Diagnosis[edit | edit source]
Diagnosis of Sézary Syndrome is based on the clinical symptoms, blood tests showing the presence of Sézary cells, and skin biopsy confirming the presence of malignant T-cells. Other tests, such as imaging studies and lymph node biopsy, may be performed to determine the extent of the disease.
Treatment[edit | edit source]
Treatment for Sézary Syndrome may involve skin-directed therapies, systemic therapies, or a combination of both. Skin-directed therapies include topical corticosteroids, phototherapy, and radiation therapy. Systemic therapies include chemotherapy, biological therapy, and stem cell transplantation.
Prognosis[edit | edit source]
The prognosis for patients with Sézary Syndrome is generally poor, with a median survival time of less than five years. However, some patients may live much longer, depending on the stage of the disease at diagnosis and the response to treatment.
See also[edit | edit source]
NIH genetic and rare disease info[edit source]
Sézary Syndrome is a rare disease.
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