title: Cutaneous tumor cell load correlates with survival in patients with Sézary syndrome.
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.
  
  
  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.
authors.affiliation: Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany. nina.booken@umm.de
authors.forename: Nina
authors.initials: N
authors.lastname: Booken
authors.forename: Jan Peter
authors.initials: JP
authors.lastname: Nicolay
authors.forename: Christel
authors.initials: C
authors.lastname: Weiss
authors.forename: Claus-Detlev
authors.initials: CD
authors.lastname: Klemke
mesh_terms: D000368:Aged; D015897:Comorbidity; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D012751:Sezary Syndrome / Q000401:mortality* / Q000628:therapy*; D012878:Skin Neoplasms / Q000401:mortality* / Q000628:therapy*; D013223:Statistics as Topic; D016019:Survival Analysis; D015996:Survival Rate; D054556:Venous Thromboembolism