PHOTOPHERESIS IN PEDIATRIC-PATIENTS WITH DRUG-RESISTANT CHRONIC GRAFT-VERSUS-HOST DISEASE

Photopheresis (ECP) is a new type of photochemotherapy, used for the t reatment of oncological and autoimmune diseases. Lymphocytes are drawn from the patients by leukapheresis, treated with 8-methoxypsoralen (8 -MOP) and ultraviolet light A (UVA) in an extracorporeal system and th en reinfused. Skin exposure to 8-MOP and UVA (PUVA) has been shown to relieve cutaneous symptoms of graft-versus-host disease (GVHD) in bone marrow transplant (BMT) recipients. ECP, which is similar in some way s to PUVA, has been used in this study to treat four paediatric patien ts who developed chronic GVHD following BMT and in whom GVHD had faile d to respond to conventional immunosuppressive therapy Following ECP, skin lesions cleared almost completely and pulmonary function tests im proved in two of three patients with cutaneous and lung involvement. S erum bilirubin and transaminases gradually normalized, and gamma GT de creased considerably in the remaining patient who had a severe cholest atic hepatopathy. The Karnofsky performance score increased to 90% in the three patients with positive responses to ECP and remained unchang ed (40%) in the patient who did not respond. Immunosuppressive therapy was reduced in three patients and eventually discontinued in two, No significant side-effects were observed during the treatment. Our resul ts suggest that ECP is a non-aggressive treatment that may benefit pat ients with chronic GVHD who do not respond to standard immunosuppressi ve therapy.