What is Sézary Syndrome?Sézary syndrome is an aggressive type of skin cancer that results from the rapid, uncontrolled division of T lymphocytes. T lymphocytes or T cells are white blood cells. They are a major component of the body’s immune system. They play a role by directly killing infected host cells, activating other immune cells, and regulating the body’s immune system.
Disease Etiology (Causes)There is no well-known cause. However, certain chromosomal abnormalities or genetic mutations (faulty alterations in the genes) are considered responsible for the development of Sézary syndrome.
Disease EpidemiologyIt is a rare disease and makes approximately 3-5% of all cutaneous T-cell lymphoma.
Signs and SymptomsThe patient may suffer from the following signs and symptoms. These are
- Itching rash that covers a large portion of the body skin
- Swollen or enlarged lymph nodes
- Hair loss (alopecia)
- Swelling or edema of the skin
- The thick skin of the palms and soles
- Outward turning of the lower eyelid
- It can also affect the spleen, liver, and bone marrow
DiagnosisThe following are the diagnostic tests and procedures that can be used to make a diagnosis. These are
- Complete blood count (CBC) with peripheral smear
- Blood coagulation profile
- Tumour biopsy
- CT- scan, MRI, PET scan, Ultrasonography (USG), and X-Ray to detect the site and extent of cancer.