Sézary Syndrome
definition
Sézary cells are cancerous T-lymphocytes. They are found in the blood, skin, and lymph nodes of patients with Sézary syndrome, an aggressive form of cutaneous T-cell lymphoma.
This lymphoma, which often spreads to other organs, accounts for approximately 3%–5% of all cutaneous T-cell lymphomas. It can affect people of any age but is more common in older adults.
The disease occurs more frequently in men, and Caucasian population is at higher risk compared to African population.
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Symptoms
Sézary syndrome is primarily characterized by erythroderma, an extensive skin redness that may cover up to 80% of the skin surface. It is accompanied by severe itching and scaling, although these symptoms may also appear in other conditions, such as psoriasis.
Common symptoms also include swelling, skin tumors, enlarged lymph nodes, abnormalities of the nails and eyelids, hair loss, and difficulty regulating body temperature.
Advanced stages of the syndrome may affect organs such as the liver and lungs, increasing the risk of developing other cancers.
Stages
Staging helps in better understanding the disease’s spread and aids in selecting appropriate treatment options. The stages are as follows:
- Stage 1: Mild skin redness.
- Stage 2: Red skin with enlarged lymph nodes, but without cancerous cells.
- Stage 3: Widespread redness, with possible presence of Sézary cells in the blood.
- Stage 4: Full skin involvement with high Sézary cell count (Stage 4A), and in Stage 4B the cancer has spread to other organs.
Etiology
The rare nature of Sézary syndrome makes it difficult to determine its exact cause. Most cases appear to be related with chromosomal abnormalities, found only in cancerous cells.
Common findings include loss of genetic material on chromosomes 10 and 17, or extra genetic material on chromosomes 8 and 17. However, it is unclear whether these changes are the cause of cancer or simply associated with the disease.
Diagnosis
Diagnosing Sézary syndrome requires a combination of diagnostic data beyond just clinical symptoms.
The physician reviews the patient’s medical history and performs specialized tests, such as skin biopsy, blood analysis, and PCR for molecular markers.
The biopsy involves sampling tissue for analysis, and may extend to lymph nodes and bone marrow. Imaging tests such as CT, MRI, and PET scans are typically performed to assess cancer spread.
The final diagnosis is confirmed through biopsy and imaging techniques.
Therapeutic Management
Treatment for cutaneous lymphoma depends on the stage of the disease, as well as the patient’s age and overall health.
First-Line Therapy
Phototherapy with psoralen and UVA (PUVA) is recommended in the patch or plaque stage and requires treatment 2–3 times per week.
UVB radiation therapy, particularly narrowband UVB, is recommended at the patch stage, as its skin penetration is not as deep as PUVA. It also requires treatment 2–3 times per week.
Imiquimod is an immune response modifier and can be applied topically as a cream during the early stage of the disease.
Second-Line Therapy
Bexarotene, a retinoid, is an antineoplastic agent and may be used in combination with PUVA or interferon-alpha and extracorporeal photopheresis (ECP).
Interferon-alpha is administered via intramuscular injection and requires high-dose treatment.
Vorinostat, a histone deacetylase (HDAC) inhibitor, may help reduce the sensation of itching.
Methotrexate is a chemotherapy agent and immunosuppressant. It is generally well tolerated and administered orally on a weekly basis.
Total skin electron beam (TSEB) radiation may reduce symptoms and the growth of cancer cells.
Local radiation therapy can be applied when large plaques or tumor nodules are detected.
Alemtuzumab acts via antibody-dependent cell-mediated cytolysis and complement-dependent cytolysis, following binding to the surface of T- and B-lymphocytes. It exerts an immunomodulatory effect by eliminating and repopulating lymphocytes.
When conventional treatments are ineffective, stem cell transplantation may be approved.