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Non-Classic Signs of Sézary Syndrome: A Review

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Abstract

The majority of patients with Sézary syndrome (SS) present with classic symptoms of erythroderma, lymphadenopathy, and pruritus. However, there have been numerous reports of patients with SS who have non-classic signs. In this review, we report the less common clinical presentations of SS and discuss their relevant treatments. Our search included all literature on SS since 2008, the year the World Health Organization (WHO) incorporated the diagnostic criteria for SS into the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. We reviewed 896 articles and identified 505 patients with non-classic presentations of SS. Of these 505 patients, the most common non-classic signs of SS were keratoderma, onychodystrophy, alopecia, leonine facies, and ectropion. Given the aggressive and highly symptomatic nature of SS, it is imperative that clinicians recognize the less common signs of the disease to prevent delays in diagnosis and treatment. To our knowledge, this is the first review of the clinical variations of SS with a focus on non-classic signs and symptoms.

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Acknowledgements

Dr. Madeleine Duvic is the Blanche Bender Professor in Cancer Research and an honoree of the Stanton CTCL Research Fund.

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No external funding was used in the preparation of this review.

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  1. Lisa Morris and Jessica Tran contributed equally to this review.

Authors and Affiliations

  1. University of Missouri-Columbia School of Medicine, Columbia, MO, USA

    Lisa Morris

  2. Baylor College of Medicine, Houston, TX, USA

    Jessica Tran

  3. Department of Dermatology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd. Unit 1452, Houston, TX, 77030, USA

    Jessica Tran & Madeleine Duvic

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Lisa Morris, Jessica Tran, and Madeleine Duvic declare that they have no conflicts of interest that might be relevant to the contents of this review.

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Morris, L., Tran, J. & Duvic, M. Non-Classic Signs of Sézary Syndrome: A Review. Am J Clin Dermatol 21, 383–391 (2020). https://doi.org/10.1007/s40257-020-00501-7

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