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

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Summary Background: Sézary syndrome (SS) is defined by the triad of erythroderma, generalized lymphadenopathy and more than 1 000 circulating Sézary cells/?l in the peripheral blood. Patients and Methods: We screened the cutaneous lymphoma registry of our department for SS patients to identify cli...

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Bibliographic Details
Main Authors: Booken, Nina (Author) , Nicolay, Jan Peter (Author) , Weiß, Christel (Author) , Klemke, Claus-Detlev (Author)
Format: Article (Journal)
Language:English
Published: 20 November 2012
In: Journal der Deutschen Dermatologischen Gesellschaft
Year: 2013, Volume: 11, Issue: 1, Pages: 67-79
ISSN:1610-0387
DOI:10.1111/j.1610-0387.2012.08027.x
Online Access:Verlag, Volltext: http://dx.doi.org/10.1111/j.1610-0387.2012.08027.x
Verlag, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1610-0387.2012.08027.x
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Author Notes:Nina Booken, Jan Peter Nicolay, Christel Weiss, Claus‐Detlev Klemke
Description
Summary:Summary Background: Sézary syndrome (SS) is defined by the triad of erythroderma, generalized lymphadenopathy and more than 1 000 circulating Sézary cells/?l 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.
Item Description:Gesehen am 12.04.2018
Physical Description:Online Resource
ISSN:1610-0387
DOI:10.1111/j.1610-0387.2012.08027.x

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