THERAPEUTIC EXPERIENCE WITH EXTRACORPOREA L PHOTOPHORESIS - TECHNICALASPECTS, MONITORING AND CLINICAL-RESULTS IN 41 PATIENTS

Extracorporeal photophoresis (ECP), a therapeutic modality that has be en under investigation for some years, is based on separation of a leu cocyte/lymphocyte-enriched cell fraction from the peripheral blood, ex tracorporeal treatment of the cells with 8-MOP/UVA and subsequent rein fusion of the cells in the patient. Its main effects seem to consist i n changes to the immunologic behaviour of the photoinactivated/modulat ed cells. The immune response of the host is obviously stimulated by t his treatment. ECP is normally performed for 4 h per day on 2 consecut ive days every 4 weeks. The treatment is well tolerated and causes few side effects. In our department, 1210 ECP treatments were administere d to 41 patients between 1990 and 1994 and a preliminary evaluation wa s 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. Patie nts with erythroderma and preserved immunocompetence achieved the best responses of all patients with CTCL. A treatment combining ECP with r lFN-alpha, PUVA and/or radiation was also successful in patients with tumour-stage CTCL and lymph node involvement. Progressive systemic scl eroderma responded in more than 50% of our cases. Treatment results we re impressive in 4 patients with chronic graft-versus-host disease pre senting with sclerodermatous and lichenoid changes of the skin and muc ous membranes. A clear improvement was also observed in the patient wi th pemphigus vulgaris refractory to standard therapies and in another patient with scleromyxoedema (Arndt-Gottron syndrome). The effectivene ss of ECP seems to be quite well established in CTCL, but remains to b e examined in autoimmune dermatoses. ECP is an attractive addition to the dermatological therapies available but our experience is still pre liminary.