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Cutaneous tumor cell load correlates with survival in patients with Sézary syndrome

Authors

  • Nina Booken,

    1. Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany
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    • Contributed equally.

  • Jan Peter Nicolay,

    1. Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany
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    • Contributed equally.

  • Christel Weiss,

    1. Department of Statistics, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany
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  • Claus-Detlev Klemke

    1. Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany
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  • Conflict of interest CDK received travel grants to attend scientific meetings and fees for scientific talks from TEVA/Cephalon pharma GmbH and Therakos, Johnson & Johnson Medical GmbH. He is a member of the TEVA cutaneous lymphoma advisory board. The other authors report no conflicts of interest.

Dr. Nina Booken, MD, Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1–3, 68135 Mannheim, Germany E-mail: nina.booken@umm.de

Summary

Background: Sézary syndrome (SS) is defined by the triad of erythroderma, generalized lymphadenopathy and more than 1 000 circulating Sézary cells/μl in the peripheral blood.

Patients and Methods: We screened the cutaneous lymphoma registry of our department for SS patients to identify clinical features of SS besides the defining criteria and to correlate them with disease survival.

Results: 24 SS patients were analyzed retrospectively. The mean age was 65 years with 62 % male patients. The median follow-up time was 32.5 months with an estimated 5-year overall survival rate of 76 %. All patients complained about itching and presented with palmoplantar keratoderma. 62.5 % had nail involvement, 21 % alopecia, 12.5 % ectropion, 4 % prurigo nodularis, 8 % localized and 8 % generalized skin tumors, including leonine facies. In addition, 33 % had infections and also 33 % had venous thromboembolism. We identified cutaneous tumor cell load as a significant prognostic marker for SS. None of the other parameters were associated with disease specific survival.

Conclusions: Clinically SS is characterized by various presentations beyond erythroderma. The cutaneous tumor cell load in SS is strongly associated with outcome and survival. We demonstrate a high risk for venous thromboembolism in SS patients who might benefit from anti-coagulation therapies.

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