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


Hair Therapy & Transplantation

Author(s): Booken N, Nicolay JP, Weiss C, Klemke CD

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Abstract 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. © The Authors | Journal compilation © Blackwell Verlag GmbH, Berlin. This article was published in J Dtsch Dermatol Ges and referenced in Hair Therapy & Transplantation

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