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Combination therapy with extracorporeal photopheresis, interferon‐α, PUVA and topical corticosteroids in the management of Sézary syndrome
Author(s) -
Booken Nina,
Weiß Christel,
Utikal Jochen,
Felcht Moritz,
Goerdt Sergij,
Klemke ClausDetlev
Publication year - 2010
Publication title -
jddg: journal der deutschen dermatologischen gesellschaft
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.463
H-Index - 60
eISSN - 1610-0387
pISSN - 1610-0379
DOI - 10.1111/j.1610-0387.2010.07319.x
Subject(s) - photopheresis , extracorporeal photopheresis , medicine , dermatology , puva therapy , lymphoma , immunology , surgery , psoriasis , transplantation , graft versus host disease
Summary 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‐α, 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.