Sézary's disease
Encyclopedia
Sézary's disease is a type of cutaneous lymphoma
that was first described by Albert Sézary. The affected cells are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides
with lymphadenopathy
. There are currently no known causes of Sézary's disease.
are T-cell lymphomas whose primary manifestation is in the skin. The disease's origin is a peripheral CD4+ T-lymphocyte, although rarer CD8+/CD4- cases have been observed. Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease. The dominant symptoms of the disease are:
of a skin lesion can be performed to rule out any other causes.
(Zolinza) is a second-line drug for cutaneous T-cell lymphoma. Treatments are often used in combination with phototherapy and chemotherapy
. No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.
Lymphoma
Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...
that was first described by Albert Sézary. The affected cells are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides
Mycosis fungoides
-External links:* * *...
with lymphadenopathy
Lymphadenopathy
Lymphadenopathy is a term meaning "disease of the lymph nodes." It is, however, almost synonymously used with "swollen/enlarged lymph nodes". It could be due to infection, auto-immune disease, or malignancy....
. There are currently no known causes of Sézary's disease.
Signs and symptoms
Sézary syndrome and mycosis fungoidesMycosis fungoides
-External links:* * *...
are T-cell lymphomas whose primary manifestation is in the skin. The disease's origin is a peripheral CD4+ T-lymphocyte, although rarer CD8+/CD4- cases have been observed. Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease. The dominant symptoms of the disease are:
- Generalized erythrodermaErythrodermaErythroderma is an inflammatory skin disease with erythema and scaling that affects nearly the entire cutaneous surface....
- LymphadenopathyLymphadenopathyLymphadenopathy is a term meaning "disease of the lymph nodes." It is, however, almost synonymously used with "swollen/enlarged lymph nodes". It could be due to infection, auto-immune disease, or malignancy....
- Atypical T-cells ("Sézary cells") in the peripheral blood
- HepatosplenomegalyHepatosplenomegalyHepatosplenomegaly is the simultaneous enlargement of both the liver and the spleen . Hepatosplenomegaly can occur as the result of acute viral hepatitis or infectious mononucleosis, or it can be the sign of a serious and life threatening lysosomal storage disease...
Diagnosis
Patients who have Sézary's disease often present with skin lesions that do not heal with normal medication. A blood test generally reveals any change in the levels of lymphocytes in the blood, which is often associated with a cutaneous T-cell lymphoma. Finally, a biopsyBiopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
of a skin lesion can be performed to rule out any other causes.
Treatment
VorinostatVorinostat
Vorinostat or suberoylanilide hydroxamic acid is a member of a larger class of compounds that inhibit histone deacetylases...
(Zolinza) is a second-line drug for cutaneous T-cell lymphoma. Treatments are often used in combination with phototherapy and chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
. No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.