BACKGROUND: Extracorporeal photopheresis (ECP) is recommended for the treatment of Sézary syndrome (SS), the leukemic variant of cutaneous T-cell lymphoma (CTCL). Several combination therapies are used to increase response rates to ECP. PATIENTS AND METHODS: We report our experience with the combination therapy of ECP, interferon-alpha, PUVA and topical corticosteroids in SS. RESULTS: The treatment outcome in 12 SS patients was retrospectively analyzed and showed an overall response rate to this combination treatment of 42 % with 4/12 patients achieving a partial remission and 1/12 patients a stable disease. The median overall survival time was 42 months. We investigated several clinical and laboratory parameters as an indicator for a response to treatment in our patient cohort. A combined analysis of the erythroderma assessment scale, WBC, LDH, CD4/CD8 ratio and the number of Sézary cells revealed that a reduction of several parameters significantly correlated with response to treatment. The parameters which correlated best with response were number of Sézary cells, CD4/CD8 ratio and WBC. CONCLUSIONS: The investigated combination therapy was effective and well-tolerated in a subgroup of SS patients but needs to be evaluated in a larger patient population.
BACKGROUND: Extracorporeal photopheresis (ECP) is recommended for the treatment of Sézary syndrome (SS), the leukemic variant of cutaneous T-cell lymphoma (CTCL). Several combination therapies are used to increase response rates to ECP. PATIENTS AND METHODS: We report our experience with the combination therapy of ECP, interferon-alpha, PUVA and topical corticosteroids in SS. RESULTS: The treatment outcome in 12 SS patients was retrospectively analyzed and showed an overall response rate to this combination treatment of 42 % with 4/12 patients achieving a partial remission and 1/12 patients a stable disease. The median overall survival time was 42 months. We investigated several clinical and laboratory parameters as an indicator for a response to treatment in our patient cohort. A combined analysis of the erythroderma assessment scale, WBC, LDH, CD4/CD8 ratio and the number of Sézary cells revealed that a reduction of several parameters significantly correlated with response to treatment. The parameters which correlated best with response were number of Sézary cells, CD4/CD8 ratio and WBC. CONCLUSIONS: The investigated combination therapy was effective and well-tolerated in a subgroup of SS patients but needs to be evaluated in a larger patient population.
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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