Sezary syndrome

Alternate names

• Sezary erythroderma
• Sezary syndrome
• Sezary's lymphoma

Definition

Sezary syndrome is an aggressive form of cutaneous T-cell lymphoma which is a group of disorders that occur when T-cells (a type of white blood cell) become cancerous and affect the skin.

Summary

• It is characterized by a widespread red rash that may cover most of the body, the presence of cancerous T cells (called Sezary cells) in the blood, and abnormally enlarged lymph nodes.
• The cancerous T cells can spread to other organs in the body, including the lymph nodes, liver, spleen, and bone marrow. In addition, affected individuals have an increased risk of developing another lymphoma or other type of cancer.
• Sézary syndrome most often occurs in adults over age 60 and usually progresses rapidly; historically, affected individuals survived an average of 2 to 4 years after development of the condition, although survival has improved with newer treatments.
• Although Sézary syndrome is sometimes referred to as a variant of another cutaneous T-cell lymphoma called mycosis fungoides, these two cancers are generally considered separate conditions.

Sézary syndrome in a 61-year-old man presenting in 1972 with unrelenting itchiness of six months’ duration. On the right is his peripheral blood film stained with Periodic Acid-Schiff (PAS) showing a neoplastic T cell (Sézary cell).

Sézary cell: pleomorphic abnormal T cell with the characteristic cerebriform nuclei (Peripheral blood - MGG stain).

Cause

• The cause of Sézary syndrome is unknown.
• Most affected individuals have one or more chromosomal abnormalities, such as the loss or gain of genetic material.
• These abnormalities occur during a person's lifetime and are found only in the DNA of cancerous cells.
• Abnormalities have been found on most chromosomes, but some regions are more commonly affected than others.
• People with this condition tend to have losses of DNA from regions of chromosomes 10 and 17 or additions of DNA to regions of chromosomes 8 and 17.
• It is unclear whether these alterations play a role in Sézary syndrome, although the tendency to acquire chromosomal abnormalities (chromosomal instability) is a feature of many cancers.
• It can lead to genetic changes that allow cells to grow and divide uncontrollably.
• Although a small percentage of cases of Sezary syndrome are associated with human T-lymphotropic viruses type 1 and type 2, the underlying cause of most cases is currently unknown.

Inheritance

• The inheritance pattern of Sézary syndrome has not been determined.
• This condition occurs in people with no history of the disorder in their family and is not thought to be inherited in most cases.

Signs and symptoms

The signs and symptoms of this condition can vary but may include:

• A red, itchy rash that covers large portions of the body
• Enlarged lymph nodes
• Alopecia (hair loss)
• Thickened skin on the palms of the hands and soles of the feet
• Abnormalities of the fingernails and toenails
• Ectropion
• Hepatosplenomegaly (enlarged liver and spleen)

Affected people may also have an increased risk of developing another lymphoma or other type of cancer.

Clinical presentation

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

80%-99% of people have these symptoms

• Abnormal lymphocyte morphology
• Cutaneous T-cell lymphoma
• Dry skin
• Erythroderma
• Lichenification
• Lymphadenopathy(Swollen lymph nodes)
• Neoplasm of the skin(Skin tumors)
• Pruritus(Itching)

30%-79% of people have these symptoms

• Alopecia(Hair loss)
• Hepatomegaly(Enlarged liver)
• Immunodeficiency(Decreased immune function)
• Nail dystrophy(Poor nail formation)
• Palmoplantar keratoderma(Thickening of palms and soles)
• Splenomegaly(Increased spleen size)

5%-29% of people have these symptoms

• Abnormal facial shape(Unusual facial appearance)
• Abnormal immunoglobulin level
• Abnormal pleuramorphology
• Ectropion(Eyelid turned out)
• Edema(Fluid retention)
• Gangrene(Death of body tissue due to lack of blood flow or infection)
• Irregular hyperpigmentation
• Peripheral neuropathy
• Skeletal muscle atrophy(Muscle degeneration)
• Tremor

Diagnosis

A diagnosis of Sezary syndrome is often suspected in people with characteristic signs and symptoms. Additional testing can then be ordered to confirm the diagnosis. This may include:

• A skin biopsy
• A complete blood count
• Peripheral blood smear
• Immunophenotyping
• T-cell receptor (TCR) gene rearrangement test
• Flow cytometry

Treatment

In general, there are six different treatment options available to people with Sezary syndrome. These include:[2]

• Photodynamic therapy
• Radiation therapy
• Chemotherapy
• Other drug therapy (i.e. topical steriods, retinoids)
• Biologic therapy
• Targeted therapy

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Mogamulizumab (Brand name: Poteligeo)mogamulizumab (Poteligeo) was approved for the treatment of adult patients with relapsed or refractory mycosis fungoides (MF) or Sézary syndrome (SS) after at least one prior systemic therapy.

Epidemiology

• Sézary syndrome is a rare condition, although its prevalence is unknown.
• It is the second most common form of cutaneous T-cell lymphoma after mycosis fungoides, accounting for approximately 3 to 5 percent of cases of cutaneous T-cell lymphoma.

NIH genetic and rare disease info

• NIH info on Sezary syndrome

Sezary syndrome is a rare disease.

Sezary syndrome Resources
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