Sézary Syndrome

Definition of Sézary Syndrome

Sézary syndrome abbreviated as SS is an erythrodermic type of cutaneous T-Cell lymphoma (CTCL) which is a term that includes all groups of cutaneous lymphomas that arise from T-lymphocytes. The cancer affects the skin and causes various types of skin abrasions. In this syndrome, the malignant T-cells are named as Sézary cells and are situated in the lymph nodes, skin, and blood.

The cells are abnormally shaped nucleus, known as cerebriform. Sézary cells originated in the rash although the skin cells are not malignant; problems occur when Sézary cells transfer into the skin from the blood. The cancerous T cells could possibly metastasize to other organs within the body such as the liver, bone marrow, lymph nodes, and spleen.

The risk of developing another type of cancer is increased for those affected individuals. Sézary syndrome is common among people who are over 60 years old and progresses rapidly. Sometimes, the syndrome is mistaken as a variety of another T-cell lymphoma known as mycosis fungoides which primarily affects the skin and is the most common type of CTCL. The two cancers are generally different from each other and are separate conditions.

Symptoms

Symptoms of patients with Sézary syndrome include:

• Red, flaky, and thickened skin
• Severe itching of the skin
• Thickened nails
• Skin firmness
• Diffuse hair loss
• Lichenification which is the increased skin blemishes due to rubbing and scratching
• Ectropion or the turning of eyelid outward far from the eyeball
• Depression
• Lack of sleep
• Anxiety
• Enlarged spleen

Other malignancies that are associated with Sézary syndrome are:

• Bladder cancer
• Hodgkin or non-Hodgkin lymphoma
• Melanoma

Causes & Pathology

Sézary syndrome is an uncommon condition and exact cause is not known. It is the second most common type of CTCL after mycosis fungoides, with an estimation of 3-5% of cases. Most individuals that are affected with the condition have one or more chromosomal abnormalities like the gain or loss of genetic material.

The changes in the genes are considered somatic since they are obtained during a person’s lifetime, are not hereditary, and only significant for the DNA of cancerous cells. Individuals with Sézary syndrome have frequent deletions of DNA from chromosomes 10 and 17 or replications of DNA chromosomes 8 and 17. Nonetheless, it is still not clear whether these changes play a role in the developing process of the condition.

Diagnosis

A diagnosis of Sézary syndrome is commonly suspected in individuals with specific signs and symptoms. Additional tests that may be performed to confirm the diagnosis include:

• Skin biopsy – A skin biopsy done by removing a small piece of skin and is sent to the laboratory to be studied under a microscope. An indurated skin is better if it is possible.

• Complete blood count (CBC) – It is where a blood sample is taken and assessed by the physician for abnormalities.

• Peripheral blood smear – A type of procedure wherein a blood sample is surveyed under a microscope to see whether the cells appear normally and to count circulating blood cells.

• Immunophenotyping – A method used to distinguish cells according to the kinds of markers or antigens on the cell surface.

• T-Cell receptor gene rearrangement test – An examination done in a laboratory where the cells of a tissue sample is scrutinized to see if there are unusual changes in the genes.

• Flow cytometry – The use of a light-sensitive dye to measure the amount of cells in a sample of blood, cell characteristics, and tumor markers that is possibly present.

Treatment

There is no standard therapy in treating Sézary syndrome. Treatment options may include:

• Photodynamic therapy – Drugs and specific type of laser light to destroy cancer cells are used.
• Radiation therapy – A treatment that aims to kill cancer cells or prevent them from growing through high-energy x-rays.
• Chemotherapy – The use of drugs to prevent the cancer cells from growing by killing them or stop them from dividing.
• Biologic therapy – This therapy makes use of patient’s own immune system to battle with cancer. A laboratory or body substances are used to restore the natural defenses of the body against cancer.

Prognosis

Sézary syndrome usually spread to other organs fast and prognosis in general is poor containing a median survival of about 2 to 4 years, although the survival chances have increased with newer treatments. 24% of 5-year survival rate had been reported. A patient might respond for a short time, but recurrence of the disease is frequently unavoidable. Some patients undergo into remission after treatment. The patient will eventually die as a result of the complications of the disease.

Pictures

Image 1

Image 2 – en.wikipedia.org

Image 3

References:

1. https://rarediseases.info.nih.gov/gard/7629/sezary-syndrome/resources/1
2. http://dermnetnz.org/dermal-infiltrative/sezary.html
3. http://ghr.nlm.nih.gov/condition/sezary-syndrome
4. Duvic, M, Talpur, R, Ni, X et al (2007). Phase 2 trial of oral vorinostat (suberoylanilide hydroxamic acid, SAHA) for refractory cutaneous T-cell lymphoma (CTCL). Blood. 109: 31–39.
5. Richardson, SK, Lin, JH, Vittorio, CC et al (2006). High clinical response rate with multimodality immunomodulatory therapy for Sézary syndrome. Clin Lymph Myeloma. 7: 226–232.
6. Foss, F, Demierre, MF, and DiVenuti, G (2005). A phase-1 trial of bexarotene and denileukin diftitox in patients with relapsed or refractory cutaneous T-cell lymphoma. Blood. 106: 454–457.