Sezary syndrome

Sezary syndrome is a rare disease in which person’s T-cells become malignant and affect the skin causing red rash.

Overview

Sezary syndrome is a rare aggressive type of cutaneous T-cells lymphomas described by Albert Sezary. Skin T-cell lymphomas are characterized by the presence of abnormal malignant T-cells (called the Sezary cells) that proliferate quickly and infiltrate the skin and lymph nodes. Moreover, Sezary syndrome compline only about 3% of all cutaneous lymphomas with an annual incidence of 1/10,000,000.

Unfortunately, at this point, the cause of Sezary syndrome remains unknown. The disorder is usually seen in individuals over the age of 60. In general prognosis for affected individuals is poor with a median survival of 5 years.

Causes

The cause of the disease is unknown, though sometimes mutations in the MYB proto-oncogene and the interleukin-22 receptor subunit alpha-2 gene (IL22RA2) may be detected. Abnormalities in chromosomes 8, 10 and 17 were also detected in individuals suffering from the Sezary syndrome. Furthermore, sometimes the disease is associated with human T-lymphotropic viruses type 1 and type 2 infection. It was discovered that the Sezary cells have increased quantities of mucopolysaccharides though the role of this substance is not established.

Symptoms

In Sezary syndrome person’s peripheral CD4+ T-cells become cancerous and start to proliferate. This enormous quantity of T-cells called the Sezary cells to infiltrate the skin and lymph nodes. The pathologic T-cells accumulate under the skin forming Pautrier’s microabscesses. Other organs such as liver, spleen and bone marrow may also be affected.  

The symptoms of the disease include:

  • Red itchy rash spread over large areas of the body known as erythroderma;
  • Skin plaques and tumors (Pautrier’s microabscesses);
  • Thick dry skin on the soles of the feet and hands that peels off and scales (palmoplantar keratoderma);
  • Skin swelling (edema);
  • Enlarged lymph nodes;
  • Enlarged liver and spleen (hepatosplenomegaly);
  • Hair loss (alopecia);
  • Fever with chills;
  • Nails malformations;
  • Ectropion (a condition when the lower eyelids turn outwards);

Staging

The following stages are recognized in Sezary syndrome:

 1A: Less than 10 percent of the skin is covered with red rash.

1B: More than 10 percent of the skin is affected.

2A: Any amount of skin is affected. Lymph nodes are enlarged, but there are no cancerous cells on biopsy.

2B: There are one or more tumors larger than 1 cm on the skin. Lymph nodes appear enlarged, however, there are no malignant cells.

3A: Most of the skin is affected and there are cutaneous tumors, plaques, or patches. Lymph nodes may be normal or enlarged, though not cancerous. The blood may contain a few Sezary cells.

3B: Most of the skin appears involved. Lymph nodes may be enlarged or of normal size. There is a low quantity of Sezary cells in the blood.

4A(1): The whole skin surface is affected. Lymph nodes may be enlarged. The number of Sezary cells in the blood is high.

4A(2): Skin lesions are observed everywhere on the skin. There are enlarged lymph nodes containing abnormal cells. Sezary cells may be detected in the blood.

4B: All of the skin surfaces have lesions. The lymph nodes may be normal or abnormal. Other organs or tissues are infiltrated with Sezary cells.

Diagnosis

  • Skin biopsy and lymph node biopsy revealed the presence of abnormal Sezary cells that have nuclei of abnormal shape (Buttock cell of cerebriform shape);
  • Periodic Acid-Schiff is used to stain peripheral blood film and detect the T-cells;
  • There is an increased number of leukocytes in complete blood cell count, namely – lymphocytes;
  • Flow cytometry is performed to detect abnormal cells and estimate their quantity. This information is helpful to define the stage of the disease, predict the outcomes and control the treatment;
  • X-ray;
  • PET-scan to detect the distribution of malignant cells;

Treatment

Sezary syndrome treatment usually includes a combination of several treatment options including chemotherapy and radiotherapy depending on the stage of the disease and person’s condition.

PUVA therapy

Psoralen is administered intravenously. This substance accumulates in the cancer cells and stimulates their destruction when the skin is exposed to ultraviolet A (UVA) light.

Extracorporeal photopheresis

Some amount of blood is withdrawn from the body and using UVA is cleared from the malignant cells and finally reintroduced to the body. 

Radiation therapy

Radiation therapy is used to destroy the cancerous cells by the X-rays.

Chemotherapy

Chemotherapeutic agents such as methotrexate, bexarotene, liposomal doxorubicin, gemcitabine, chlorambucil, cyclophosphamide are used to treat Sezary syndrome.

Biologic therapy

There is also a bunch of novel medications that may be used in Sezary syndrome treatment that include interferons and monoclonal antibodies (alemtuzumab, brentuximab). These drugs are aimed to assist a person’s own immune system to fight cancer.

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