BACKGROUND: Extracorporeal photopheresis (ECP) is an effective treatment for cutaneous T-cell lymphoma. Controversy has arisen regarding its ability to improve survival rates in Sézary syndrome (SS). We describe our experience with ECP in the treatment of SS and erythrodermic mycosis fungoides, with particular emphasis on early predictors of long-term outcome. OBSERVATIONS: We included 17 patients (15 with SS and 2 with erythrodermic mycosis fungoides) who received ECP as initial treatment. Four of these patients were moribund on presentation (Eastern Cooperative Oncology Group Performance Status score, 4) and underwent only 1 to 2 cycles of ECP. The median survival was 56 months for the 11 patients with SS and an Eastern Cooperative Oncology Group Performance Status score of less than 4. If all 15 patients with SS are considered, median survival was 34 months. Response after 5 months of ECP correlated with long-term survival. A low number (<6.0 x10(3)/ micro L) of circulating CD4(+)CD7(-) lymphocytes correlated with response after 5 months of ECP. CONCLUSIONS: Extracorporeal photopheresis is a safe, effective, and well-tolerated treatment for erythrodermic mycosis fungoides and SS. Low numbers of CD4(+)CD7(-) cells in the circulation and a positive response after 5 months of therapy predicted long-term survival. Moribund patients are much less likely to benefit from ECP.
BACKGROUND: Extracorporeal photopheresis (ECP) is an effective treatment for cutaneous T-cell lymphoma. Controversy has arisen regarding its ability to improve survival rates in Sézary syndrome (SS). We describe our experience with ECP in the treatment of SS and erythrodermic mycosis fungoides, with particular emphasis on early predictors of long-term outcome. OBSERVATIONS: We included 17 patients (15 with SS and 2 with erythrodermic mycosis fungoides) who received ECP as initial treatment. Four of these patients were moribund on presentation (Eastern Cooperative Oncology Group Performance Status score, 4) and underwent only 1 to 2 cycles of ECP. The median survival was 56 months for the 11 patients with SS and an Eastern Cooperative Oncology Group Performance Status score of less than 4. If all 15 patients with SS are considered, median survival was 34 months. Response after 5 months of ECP correlated with long-term survival. A low number (<6.0 x10(3)/ micro L) of circulating CD4(+)CD7(-) lymphocytes correlated with response after 5 months of ECP. CONCLUSIONS: Extracorporeal photopheresis is a safe, effective, and well-tolerated treatment for erythrodermic mycosis fungoides and SS. Low numbers of CD4(+)CD7(-) cells in the circulation and a positive response after 5 months of therapy predicted long-term survival. Moribund patients are much less likely to benefit from ECP.
Authors: Laura Y McGirt; Christopher Thoburn; Allan Hess; Eric C Vonderheid Journal: Photodermatol Photoimmunol Photomed Date: 2010-08 Impact factor: 3.135
Authors: Minh Lam; Yoojin Lee; Min Deng; Andrew H Hsia; Kelly A Morrissey; Chunlin Yan; Kashif Azzizudin; Nancy L Oleinick; Thomas S McCormick; Kevin D Cooper; Elma D Baron Journal: Adv Hematol Date: 2010-12-12
Authors: Arun Alfred; Peter C Taylor; Fiona Dignan; Khaled El-Ghariani; James Griffin; Andrew R Gennery; Denise Bonney; Emma Das-Gupta; Sarah Lawson; Ram K Malladi; Kenneth W Douglas; Tracey Maher; Julie Guest; Laura Hartlett; Andrew J Fisher; Fiona Child; Julia J Scarisbrick Journal: Br J Haematol Date: 2017-02-21 Impact factor: 6.998
Authors: R Knobler; P Arenberger; A Arun; C Assaf; M Bagot; G Berlin; A Bohbot; P Calzavara-Pinton; F Child; A Cho; L E French; A R Gennery; R Gniadecki; H P M Gollnick; E Guenova; P Jaksch; C Jantschitsch; C Klemke; J Ludvigsson; E Papadavid; J Scarisbrick; T Schwarz; R Stadler; P Wolf; J Zic; C Zouboulis; A Zuckermann; H Greinix Journal: J Eur Acad Dermatol Venereol Date: 2020-10-06 Impact factor: 6.166