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

• Verfasst von: Booken, Nina [VerfasserIn]
• Verfasst von: Nicolay, Jan Peter [VerfasserIn]
• Verfasst von: Weiß, Christel [VerfasserIn]
• Verfasst von: Klemke, Claus-Detlev [VerfasserIn]
• Titel: Cutaneous tumor cell load correlates with survival in patients with Sézary syndrome
• Verf.angabe: Nina Booken, Jan Peter Nicolay, Christel Weiss, Claus‐Detlev Klemke
• E-Jahr: 2012
• Jahr: 20 November 2012
• Umfang: 13 S.
• Fussnoten: Gesehen am 12.04.2018
• Titel Quelle: Enthalten in: Deutsche Dermatologische GesellschaftJournal der Deutschen Dermatologischen Gesellschaft
• Ort Quelle: Berlin : Wiley-Blackwell, 2003
• Jahr Quelle: 2013
• Band/Heft Quelle: 11(2013), 1, Seite 67-79
• ISSN Quelle: 1610-0387
• DOI: doi:10.1111/j.1610-0387.2012.08027.x
• Datenträger: Online-Ressource
• Sprache: eng
• Bibliogr. Hinweis: Erscheint auch als : Druck-Ausgabe: Cutaneous tumor cell load correlates with survival in patients with Sézary syndrome. - 2012
• K10plus-PPN: 1571942688

Abstract

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.