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

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 -