Cutaneous tumor cell load correlates with survival in patients with Sézary syndrome
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Cutaneous tumor cell load correlates with survival in patients with Sézary syndrome. / Booken, Nina; Nicolay, Jan Peter; Weiss, Christel; Klemke, Claus-Detlev.
In: J DTSCH DERMATOL GES, Vol. 11, No. 1, 01.2013, p. 67-79.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Cutaneous tumor cell load correlates with survival in patients with Sézary syndrome
AU - Booken, Nina
AU - Nicolay, Jan Peter
AU - Weiss, Christel
AU - Klemke, Claus-Detlev
N1 - © The Authors | Journal compilation © Blackwell Verlag GmbH, Berlin.
PY - 2013/1
Y1 - 2013/1
N2 - 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.
AB - 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.
KW - Aged
KW - Comorbidity
KW - Female
KW - Germany/epidemiology
KW - Humans
KW - Incidence
KW - Male
KW - Middle Aged
KW - Risk Factors
KW - Sezary Syndrome/mortality
KW - Skin Neoplasms/mortality
KW - Statistics as Topic
KW - Survival Analysis
KW - Survival Rate
KW - Venous Thromboembolism
U2 - 10.1111/j.1610-0387.2012.08027.x
DO - 10.1111/j.1610-0387.2012.08027.x
M3 - SCORING: Journal article
C2 - 23167557
VL - 11
SP - 67
EP - 79
JO - J DTSCH DERMATOL GES
JF - J DTSCH DERMATOL GES
SN - 1610-0379
IS - 1
ER -