Sezary syndrome is a type of cutaneous T-cell lymphoma, where lymphocytes (white blood cells) become cancerous and attack the skin, blood, and at times the lymph nodes. This disease is common among middle-aged and older people. Sezary syndrome progresses rapidly once it develops. When these lymphocytes develop in the blood, they are called Sezary cells and when a person has a high level of these cells in their blood, they have Sezary syndrome. Photopheresis and immunotherapy are treatment options that may help control the effects of this condition, but there is no cure. Sezary syndrome is known to increase your risk of cancer.

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Sezary syndrome

What Is Sezary Syndrome?

Sézary syndrome is a rare type of cancer that affects the skin and the blood, which can result in

  • Tumours cells in the blood
  • Enlarged lymph nodes
  • Extensive skin rashes or lesions

It is a chronic cutaneous T-cell lymphoma (CTCL), which causes the white blood cells called T cells to mutate out of control. Mycosis fungoides another form of CTCLs usually affects the skin in the form of a red rash. However, in Sezary syndrome cancerous lymphocytes spread from the skin to the blood and travel to the lymph nodes and other regions of the body.

What Causes Sézary Syndrome?

The exact cause of Sezary syndrome is still not clear. A few people with the disease may have a human T-cell leukemia virus, which affects lymphocytes. These viruses contain alterations to DNA, but they are not inherited.

Symptoms Of Sézary Syndrome

This syndrome can cause several signs and changes in the skin including:

  • Dry or itchy skin
  • Red rash over the body
  • Skin tumors
  • Thickening of the palms of the hands and soles of the feet
  • Other symptoms may include:
  • Abnormal nail growth
  • Alopecia
  • Edema
  • Enlarged liver
  • Enlarged spleen
  • Fever
  • Problems regulating body temperature
  • Swollen lymph nodes
  • Sudden weight loss

Also Read: Leukemia: Causes, Symptoms and Treatment

Diagnosis

Doctors specialized in treating lymphomas can diagnose Sezary Syndrome, as it’s a rare disease, a hematopathologist or dermatopathologist will confirm the diagnosis. Your healthcare provider will:

  • Monitor your symptoms
  • Do a complete physical examination
  • Reviews your past medical history

If they suspect Sezary syndrome, then they may recommend doing the following tests:

Biopsy:  Biopsy of the patient’s skin, lymph nodes, or bone marrow, where a sample of tissue is tested for signs of cancer.

Blood Works: A complete blood count and peripheral blood smear to determine red and white blood cells, platelets, and haemoglobin. Blood flow cytometry can detect cancerous cells in a patient’s blood.

Immunophenotyping: A blood or tissue sample can reveal markers present on cell surfaces, that indicate specific types of lymphomas.

T-cell receptor (TCR) gene rearrangement test: A blood or bone marrow test looks for issues in the genes that regulate T-cell function.

Treatment

Palliative therapy is generally the treatment option for Sezary syndrome, where treatment helps to lessen symptoms, but it doesn’t cure the syndrome.

The doctor recommends treatment based on the stage of cancer and its symptoms, which include:

Extracorporeal Photopheresis (ECP): Photopheresis gets rid of blood from the system and targets cancerous cells with a drug that makes them sensitive to light. The UV light then kills the harmful tumour cells before blood travels back to the system.

Immunotherapy: Immunotherapy triggers the body’s immune system to combat cancer cells.

Drug Therapies:  Certain medicated creams or ointments are prescribed to ease red, irritated skin. Retinoid creams can slow cancer cell growth.

UV Phototherapy: Ultraviolet light in the B range (UVB) or A range (UVA) is mostly used. A specialized therapy called PUVA, where the patient is given a drug called psoralen and further exposed to UVA phototherapy is at times used.

Radiation Therapy: Total skin electron beam radiation therapy is given, which offers powerful doses of radiation to the entire surface of your skin.

Targeted Therapy: In this therapy drugs are used to target proteins or genes that control how cancer cells grow and survive.

Chemotherapy: Chemotherapy may be given topically (on the skin) or systemically (throughout your body) usually as a pill or through an IV.

Prevention

This syndrome doesn’t have a clear cause, so there’s no possible way to avert the disease. However, one can lessen the risk by avoiding the human T-lymphotropic virus, where the virus spreads through:

  • Blood transfusions
  • Breastfeeding
  • Sexual contact
  • Sharing needles