Early clinical manifestations of Sézary syndrome: A multicenter retrospective cohort study - 14/09/17
Abstract |
Background |
Classic Sézary syndrome (SS) is defined by erythroderma, generalized lymphadenopathy, and leukemic blood involvement. Clinical observations suggest that SS begins as a nonerythrodermic disease.
Objective |
To describe the early clinical characteristics of patients with SS.
Methods |
A retrospective, multicenter chart review was performed for 263 confirmed cases of SS diagnosed during 1976-2015.
Results |
Erythroderma was the earliest recorded skin sign of SS in only 25.5% of cases, although most patients (86.3%) eventually developed erythroderma. In patients without erythroderma during their initial visit, the first cutaneous signs of SS were nonspecific dermatitis (49%), atopic dermatitis-like eruption (4.9%), or patches and plaques of mycosis fungoides (10.6%). The mean diagnostic delay was 4.2 years overall, 2.2 years for cases involving erythroderma at the initial presentation, and 5.0 years for cases not involving erythroderma at the initial presentation.
Limitations |
This study is retrospective.
Conclusion |
Erythroderma is uncommon as an initial sign of SS. Early SS should be considered in cases of nonerythrodermic dermatitis that is refractory to conventional treatments. In these cases, examination of the blood by PCR for monoclonal T-cell receptor rearrangement and by flow cytometry to identify an expanded or aberrant T-cell population should be considered.
Le texte complet de cet article est disponible en PDF.Key words : cutaneous T-cell lymphoma, diagnosis, diagnostic delay, erythroderma, nonerythroderma, prognosis, Sézary syndrome, survival
Abbreviations used : AD, E-MF, EORTC, ISCL, MF, SD, SS, TCR
Plan
Funding sources: Supported by the Swiss National Science Foundation (PMPDP3_151326 to EG) and the Hochspezialisierte Medizin Schwerpunkt Immunologie, Switzerland. |
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Conflicts of interest: Dr Mangold is a clinical investigator for Novartis, Eli Lilly and Company, DUSA Pharmaceuticals Inc, and Acetilion and served as an advisor for Castle Biosciences. Dr Gniadecki previously served on the advisory boards of Therakos Inc, Novartis, AbbVie Inc, and Janssen Pharmaceutical. Other authors declared no conflicts of interest. |
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Previously presented: This research was presented at the 3rd World Congress of Cutaneous Lymphomas in New York, New York, October 26-28, 2016. |
Vol 77 - N° 4
P. 719-727 - octobre 2017 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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