Other Names for this Disease
- Sézary syndrome
- Sezary's lymphoma
What causes Sezary syndrome?
Is Sezary syndrome inherited?
How might Sezary syndrome be treated?
A closely related condtion, mycosis fungoides (MF), has been reported to occur in more than one family member in several families. Sezary syndrome has been considered a "leukemic variant" of MF in the past. However, although there are conflicting reports in the literature, they are now more commonly thought to be separate conditions.
- Topical corticosteroids.
- Topical chemotherapy with mechlorethamine (nitrogen mustard) or carmustine (BCNU).
- Psoralen and ultraviolet A radiation (PUVA).
- Ultraviolet B radiation (UVB).
- Total-skin electron-beam radiation (TSEB).
- Radiation of symptomatic skin lesions.
- Interferon-alpha alone or in combination with topical therapy.
- Single-agent and multiagent chemotherapy.
- Bexarotene (topical gel or oral); retinoids.
- Denileukin diftitox (recombinant fusion protein of diptheria toxin fragments and interleukin-2 sequences).
- Combined modality treatment.
These types of treatments are aimed at producing remissions and improving symptoms. Treatment, therefore, is considered palliative for most patients. Survival in excess of 8 years is common. Patients with Sezary syndrome may benefit from participation in clinical trials evaluating new approaches to treatment.
More details about treatment of Sezary syndrome can be accessed by clicking here.
- Cutaneous T-Cell Lymphomas. NORD. January 3, 2007; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1124/viewAbstract. Accessed 4/30/2012.
- Vanessa Ngan. Cutaneous T-cell lymphoma. DermNet NZ. June 29, 2011; http://www.dermnetnz.org/dermal-infiltrative/cutaneous-t-cell-lymphoma.html. Accessed 4/30/2012.
- Hwang ST, Janik JE, Jaffe ES, Wilson WH. Mycosis fungoides and Sézary syndrome. Lancet. March 15, 2008; 371(9616):945-957.
- Hodak E. et al. Familial mycosis fungoides: report of 6 kindreds and a study of the HLA system. J Am Acad Dermatol. March 2005; 52(3 Pt 1):393-402.
- Mycosis Fungoides and Sezary Syndrome Treatment (PDQ) - Treatment Option Overview. National Cancer Institute (NCI). 2010; http://www.cancer.gov/cancertopics/pdq/treatment/mycosisfungoides/HealthProfessional/page4. Accessed 2/8/2011.