Clinical and biological analysis of Sézary syndrome: A report of four cases

Zineb Nassiri 1, 2, *, Maryem Tarmidi 1, 2, Hicham Yahyaoui 1, 2, Mustapha Ait Ameur 1, 2 and Mohamed Chakour 1, 2

1 Hematology Department, Avicenna Military Hospital, Marrakesh, Morocco.
2 Faculty of medicine and pharmacy, Cadi Ayyad University Marrakesh, Morocco.
 
Research Article
GSC Advanced Research and Reviews, 2023, 14(03), 219–223.
Article DOI: 10.30574/gscarr.2023.14.3.0087
Publication history: 
Received on 07 February 2023; revised on 14 March 2023; accepted on 17 March 2023
 
Abstract: 
Background: Sézary syndrome (SS) is a rare and aggressive type of cutaneous T cell lymphoma characterized by an intensely pruritic, exfoliative rash, known as erythroderma, with cutaneous and systemic dissemination of clonal CD4+ T cells into the blood and lymph nodes. We report through a series of 4 cases, the experience of the hematology laboratory in the diagnosis of the syndrome of Sézary.
Methods: Four patients with SS were identified retrospectively among patients with cutaneous T-cell lymphoma followed up in the dermatology department and diagnosed in the hematology laboratory of the Avicenne military hospital in Marrakesh.
Results: Four patients with SS were described: three men and one woman, mean age at diagnosis 62 years (55-71). All the patients showed generalized dry erythroderma, pruritus and lymphadenopathy. Palmo-plantar hyperkeratosis, nail lesions and alopecia were also present. The white blood cell count was elevated (>10,000 WBC/ mm3) in all 4 patients with a mean value of 17,276 and one patient among these showed an elevation of eosinophils (> 500/ µl). The blood smear showed the presence of 65% of small to medium-sized cells with a high nucleocytoplasmic ratio and cerebriform nuclei typical of Sézary cells and suggests the diagnosis of SS.
Conclusion: Sézary syndrome is a rare subtype of cutaneous T-cell lymphoma characterized by erythroderma, circulating neoplastic T cells, and poor prognosis. Microscopic findings must be correlated with the clinical presentation to make the diagnosis.
 
Keywords: 
Sézary syndrome; Lymphoma; T-cell; Cerebriform lymphocytes; Mycosis fungoides
 
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Copyright information: 
Copyright © 2023 Author(s) retain the copyright of this article. This article is published under the terms of the Creative Commons Attribution Liscense 4.0

 

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