Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.

Eyes front and ears open. Class is now in session.

The disease that we will be learning about today is:

Sézary Syndrome

Also known as Sézary disease.

What is Sézary Syndrome?

• Sézary syndrome is a form of cutaneous T-cell lymphoma
• The defining characteristic of the syndrome is the presence of pathological levels of mucopolysaccharides in the affected T-cells
  • These T-cells are called Lutzner cells or Sezary’s cells
• Some authorities regard Sézary syndrome as a late stage of mycosis fungoides (the most common type of cutaneous T-cell lymphoma) which includes enlarged lymph nodes
• Overall, the presentation of the disease is largely similar to mycosis fungoides aside from a few key differences, such as:
  • Increased lymph node involvement
  • More monotonous cellular infiltrates
  • Absence of epidermotropism (not in all cases)
• The disease is named after Albert Sézary, a French syphilogist and dermatologist who first described the illness
• The syndrome is rare, with an incidence rate of 3 in 1,000,000 people

How Do You Get It?

• Very little is known about the cause of Sézary syndrome or other forms of cutaneous T-cell lymphoma. Genetic mutations, potentially as a result of a virus, may play a role
• Sézary syndrome is twice as common in males; average age of diagnosis is between 55-60 years

What Are the Symptoms?

• The predominant signs and symptoms of Sézary syndrome include:
  • Enlarged spleen and liver
  • Redness of the skin
  • Enlarged, swollen lymph nodes
  • The detection of abnormal, cancerous T-cells in the peripheral blood
  • Thicken palms and soles of the feet

How Is It Treated?

• A number of treatment approaches may be used for Sézary syndrome, including radiation therapy, biologic therapy, chemotherapy, and photodynamic therapy
• No single approach appears to offer a distinct advantage over another; there is still an urgent need for more effective treatment options
• Chemotherapy is rarely used on its own
  • Agents used for Sézary syndrome include vorinostat and romidepsin​ 
• A number of radiation therapy approaches may be considered, such as total skin electron therapy
• Mogamulizumab was approved to treat the disease in the US and Japan

Where Can I Learn More???

• Check out our cornerstone on this disease here.