Sézary Syndrome corresponds to 3% of all cutaneous lymphomas, and it is characterized by a triad of manifestations: erythrodermia with pruritus, limphonodomegalia and atypical circulating lymphocytes (referred to as Sézary or Lutzner cells). Associated clinical manifestations include lagophthalmos, alopecia, palmoplantar hyperkeratosis and onycodystrophy. Erythrodermia may be the progression of previous patches and plaques, developing from idiopathic erythrodermia or emerging de novo.
Diagnostic Criteria in Sézary’s Syndrome (SS)
The diagnostic criteria for the syndrome, as recommended by ISCL-EORTC and countersigned in the WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues, are that the circulating monoclonal lymphocyte population should be identified by molecular or cytogenetic methods and there should be an identity between the circulating T-lymphocyte clone and the clone presented in the skin, in addition to one of the following: at least 1,000 Sézary cells per mm3 of peripheral blood, an increased population of CD4+/CD7- in peripheral blood with remarkable predominance of CD4+ cells in relation to CD8+ (CD4/CD8 ratio > 10), Sézary cells with a diameter > 14 um representing > 20% of the circulating lymphocytes and, some markers like CD2, CD3, CD4 and CD5 must be absent.
The staging system used for mycosis fungoides (MF) is also used for SS, and by definition the syndrome is classified in stage III from the beginning. SS prognosis is poor, with a mean survival of 2 to 4 years. Both MF and SS patients have an increased chance of developing a second malignant neoplasia and even a second lymphoma. The opposite also seems to be true, that is, patients with a type-B lymphoma may develop type-T MF or SS more frequently than the general population.
References:
- Willemze R, van Vloten WA, Hermans J, Damsteeg MJ, Meijer CJ. Diagnostic criteria in Sézary’s syndrome: a multiparameter study of peripheral blood lymphocytes in 32 patients with erythroderma. J Invest Dermatol. 1983 Nov;81(5):392-7. [Medline]
- Hwang ST, Janik JE, Jaffe ES, Wilson WH. Mycosis fungoides and Sézary syndrome. Lancet. 2008 Mar 15;371(9616):945-57. [Medline]
- Vonderheid EC, Bernengo MG. The Sézary syndrome: hematologic criteria. Hematol Oncol Clin North Am. 2003 Dec;17(6):1367-89, [Medline]
- Yamashita T, Abbade LP, Marques ME, Marques SA. Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update. An Bras Dermatol. 2012 Nov-Dec;87(6):817-28 [Medline]
Created Jan 15, 2015.
