Sezary's lymphoma

S zary's Lymphoma S zary's Lymphoma, also known as S zary syndrome, is a rare and aggressive form of cutaneous T-cell lymphoma (CTCL). It is characterized by the presence of cancerous T-cells, known as S zary cells, in the skin, lymph nodes, and peripheral blood. This condition is named after Albert S zary, a French dermatologist who first described the disease.

Clinical Presentation

Patients with S zary's Lymphoma typically present with a triad of symptoms: 1. Erythroderma: A widespread reddening of the skin, often accompanied by scaling and itching. 2. Lymphadenopathy: Swelling of the lymph nodes. 3. Presence of S zary cells: Abnormal T-cells in the blood. Other symptoms may include alopecia, nail dystrophy, and palmoplantar keratoderma.

Diagnosis

The diagnosis of S zary's Lymphoma involves several steps:

• Skin Biopsy: To examine the presence of atypical T-cells in the skin.
• Blood Tests: To detect S zary cells in the peripheral blood.
• Flow Cytometry: To analyze the immunophenotype of the T-cells.
• Molecular Studies: To identify clonal T-cell receptor gene rearrangements.

Pathophysiology

S zary's Lymphoma is a malignancy of CD4+ T-cells. The exact cause is unknown, but it involves genetic mutations that lead to the proliferation of malignant T-cells. These cells express certain markers such as CD4, CD7, and CD26, which can be used for diagnostic purposes.

Treatment

Treatment options for S zary's Lymphoma are varied and depend on the stage and severity of the disease. They may include:

• Photopheresis: A procedure that treats the blood with ultraviolet light.
• Systemic Therapies: Such as retinoids, interferons, and histone deacetylase inhibitors.
• Chemotherapy: For more advanced cases.
• Stem Cell Transplantation: In selected patients.

Prognosis

The prognosis for S zary's Lymphoma is generally poor, with a median survival of 2-4 years after diagnosis. However, early detection and treatment can improve outcomes.

Research and Future Directions

Ongoing research is focused on understanding the genetic and molecular basis of S zary's Lymphoma, which may lead to the development of targeted therapies. Clinical trials are also exploring new treatment modalities.

See Also

• Cutaneous T-cell lymphoma
• Mycosis fungoides
• Lymphoma

References

- ,

 S zary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC), 
 Journal of Clinical Oncology, 
 2011, 
 Vol. 29(Issue: 32), 
 pp. 4210 4219,

- ,

 World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours, 
  
 IARC Press, 
 2006,

NIH genetic and rare disease info

• NIH info on Sezary's lymphoma

Sezary's lymphoma is a rare disease.