SÉZARY SYNDROME
SÉZARY SYNDROME ICD-10: L84.1
• Sézary syndrome is a rare special variant of CTCL characterized by erythroderma, and cellular infiltrates of atypical lymphocytes (Sézary cells) in the skin and in the blood.
• The disease may arise de novo or, less commonly, result from extension of a preexisting circumscribed MF. It usually occurs in patients >60 years and more commonly in males than in females.
• Patients appear sick. There is generalized scaling erythroderma with considerable thickening of the skin. Because of the bright red color, the syndrome has been called the “red man syndrome” (see Section 8 and Fig. 8-3). There is diffuse hyperkeratosis of the palms and soles, diffuse hair loss that can lead to baldness, and generalized lymphadenopathy.
• Dermatopathology: The same as MF but it may be much more subtle. The lymph nodes may contain nonspecific inflammatory cells (dermatopathic lymphadenopathy) or there can be a complete replacement of the nodal pattern by Sézary cells. The cell infiltrates in the viscera are the same as are present in the skin. Immunophenotyping: CD4+ T cells; T-cell receptor rearrangement: monoclonal process. There may be a moderate leukocytosis or a normal WBC. The buffy coat contains from 15% to 30% atypical lymphocytes (Sézary cells).
• Diagnosis rests on three features: Erythroderma, clonality in the blood, and the presence of increased numbers of atypical lymphocytes in skin tissue and in the buffy coat.
• Note that any exfoliative dermatitis can mimic Sézary syndrome (see Section 8).
• Without treatment, the course is progressive and patients die from opportunistic infections. Management is as above in MF, plus PUVA, alemtuzumab and appropriate supportive measures required for erythroderma (see Section 8).