Therapeutische Erfahrungen mit der Extrakorporalen Photopherese Technisches Vorgehen, Überwachung und klinische Ergebnisse bei 41 Hautkranken

Abstract

Extracorporeal photophoresis (ECP), a therapeutic modality that has been under investigation for some years, is based on separation of a leucocyte/lymphocyte-enriched cell fraction from the peripheral blood, extracorporeal treatment of the cells with 8-MOP/UVA and subsequent reinfusion of the cells in the patient. Its main effects seem to consist in changes to the immunologic behaviour of the photoinactivated/modulated cells. The immune response of the host is obviously stimulated by this treatment. ECP is normally performed for 4 h per day on 2 consecutive days every 4 weeks. The treatment is well tolerated and causes few side effects. In our department, 1210 ECP treatments were administered to 41 patients between 1990 and 1994 and a preliminary evaluation was performed. These patients included 21 with cutaneous T-cell lymphoma (CTCL), 10 with progressive systemic scleroderma, 4 with chronic graft-versus-host disease and 1 each with pemphigus vulgaris, epidermolysis bullosa acquisita, lupus erythematosus and cutaneous mucinosis. Patients with erythroderma and preserved immunocompetence achieved the best responses of all patients with CTCL. A treatment combining ECP with rIFN-alpha, PUVA and/or radiation was also successful in patients with tumour-stage CTCL and lymph node involvement. Progressive systemic scleroderma responded in more than 50% of our cases. Treatment results were impressive in 4 patients with chronic graft-versus-host disease presenting with sclerodermatous and lichenoid changes of the skin and mucous membranes. A clear improvement was also observed in the patient with pemphigus vulgaris refractory to standard therapies and in another patient with scleromyxoedema (Arndt-Gottron syndrome). The effectiveness of ECP seems to be quite well established in CTCL, but remains to be examined in autoimmune dermatoses. ECP is an attractive addition to the dermatological therapies available but our experience is still preliminary.

Extracorporeal Photopheresis of Cutaneous T-Cell Lymphoma Is Associated with Reduction of Peripheral CD4+ T Lymphocytes

“…ECP has been shown to cause clinical improvement and prolonged survival in patients with erythrodermic cutaneous T-cell lymphoma in the absence of limiting side effects [1, 2, 3, 4, 5]. This finding is remarkable since it has been shown that overall survival of patients with cutaneous T-cell lymphoma was not altered by conventional X-ray treatment or chemotherapy [5, 8].…”

“…Regimens combining ECP with interferon α (IFN-α), psoralen photochemotherapy and/or radiation were also successful in patients with tumor stage cutaneous T-cell lymphoma and lymph node involvement [4]. In addition to the clinical improvement a long-term, disease-free remission could be induced by ECP in a minority of patients [5].…”

Management of Severe Scleroderma with Long-Term Extracorporeal Photopheresis

“…The entire ECP procedure performed in this study has been described in detail elsewhere [18]. ECP was carried out at 4-week intervals in 2 sessions on 2 successive days (corresponding to one ECP cycle).…”

Regulationstherapie: Behandlung mit unspezifischen Reizen in der Dermatologie aus traditioneller und moderner Sicht

“…Eine modifizierte Eigenbluttherapie wurde in den 90er Jahren mit thrombozyta Èren Wachstumsfaktoren (PDWHF, plateled-derived wound healing factor) in der bioregulativen Wundheilungstherapie eingesetzt [10]. Zu den weiteren modernen Eigenbluttherapien geho È rt die extrakorporale Photophorese (ECP), die wahrscheinlich eine Modifikation der T-Zell-Rezeptoren bewirkt [11].…”