Combination therapy with extracorporeal photopheresis, interferon-alpha, PUVA and topical corticosteroids in the management of Sézary syndrome.
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.


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.
Booken
Nina
N
Department of Dermatology, Venereology and Allergology, University Medical Centre Mannheim, Ruprecht-Karls-University of Heidelberg, Mannheim, Germany. nina.booken@umm.de
Weiss
Christel
C
Utikal
Jochen
J
Felcht
Moritz
M
Goerdt
Sergij
S
Klemke
Claus-Detlev
CD
D000287:Administration, Topical; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage*; D000368:Aged; D000369:Aged, 80 and over; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D016898:Interferon-alpha / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D011701:PUVA Therapy / Q000379:methods; D017893:Photopheresis / Q000379:methods*; D012751:Sezary Syndrome / Q000188:drug therapy* / Q000473:pathology*; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D016898:Interferon-alpha