Sézary syndrome is an aggressive form of a type of blood cancer called cutaneous T-cell lymphoma. Cutaneous T-cell lymphomas occur when certain white blood cells, called T cells, become cancerous; these cancers characteristically affect the skin, causing different types of skin lesions. In Sézary syndrome, the cancerous T cells, called Sézary cells, are present in the blood, skin, and lymph nodes. A characteristic of Sézary cells is an abnormally shaped nucleus, described as cerebriform.
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.
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.
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.
Christiane Querfeld is a Dermatologist in Duarte, California. Dr. Querfeld has been practicing medicine for over 28 years and is rated as an Elite doctor by MediFind in the treatment of Sezary Syndrome. She is also highly rated in 11 other conditions, according to our data. Her top areas of expertise are Mycosis Fungoides, Cutaneous T-Cell Lymphoma, Sezary Syndrome, and Lymphomatoid Papulosis. She is board certified in Dermatology and licensed to treat patients in California and Illinois.
Jasmine Zain is a Hematologist and an Oncologist in Duarte, California. Dr. Zain has been practicing medicine for over 35 years and is rated as an Elite doctor by MediFind in the treatment of Sezary Syndrome. She is also highly rated in 18 other conditions, according to our data. Her top areas of expertise are Peripheral T-Cell Lymphoma, Anaplastic Large Cell Lymphoma, Non-Hodgkin Lymphoma, and Cutaneous T-Cell Lymphoma. She is board certified in Hematology and licensed to treat patients in California and New York.
Martine Bagot is in Paris, France. Bagot is rated as an Elite expert by MediFind in the treatment of Sezary Syndrome. She is also highly rated in 69 other conditions, according to our data. Her top areas of expertise are Cutaneous T-Cell Lymphoma, Sezary Syndrome, Mycosis Fungoides, and Non-Hodgkin Lymphoma.
Published Date:updated Last, May
Published By: National Institutes of Health