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Early clinical manifestations of Sézary syndrome: a multicenter retrospective cohort study


Mangold, Aaron R; Thompson, Agnieszka K; Davis, Mark D; Saulite, Ieva; Cozzio, Antonio; Guenova, Emmanuella; Hodak, Emmilia; Amitay-Laish, Iris; Pujol, Ramon M; Pittelkow, Mark R; Gniadecki, Robert (2017). Early clinical manifestations of Sézary syndrome: a multicenter retrospective cohort study. Journal of the American Academy of Dermatology, 77(4):719-727.

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.

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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Dermatology Clinic
Dewey Decimal Classification:610 Medicine & health
Uncontrolled Keywords:Sézary syndrome; cutaneous T-cell lymphoma; diagnosis; diagnostic delay; erythroderma; nonerythroderma; prognosis; survival
Language:English
Date:2017
Deposited On:21 Dec 2017 14:21
Last Modified:19 Feb 2018 09:42
Publisher:Elsevier
ISSN:0190-9622
OA Status:Closed
Publisher DOI:https://doi.org/10.1016/j.jaad.2017.05.036
PubMed ID:28709694

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