Graft versus host disease (GVHD) is a major complication of haematopoietic stem-cell transplantation (HSCT). Historically, GVHD occurring within the first 100 days post HSCT was classified as acute GVHD (aGVHD). More recently, the National Institutes of Health (NIH) consensus criteria for clinical trials proposed that clinical manifestations rather than time after transplantation should be used to distinguish acute from chronic GVHD and introduced the term late acute GVHD, which includes persistent, recurrent, or late-onset aGVHD.1 Clinical manifestations of aGVHD involve the skin, gastrointestinal tract and liver.2 The incidence of aGVHD is reported to range from 10 to 80 %, depending on risk factors such as the use of unrelated donors, total body irradiation and transplant conditioning regimen.3 Acute GVHD is, in turn, a strong risk factor for transplant-related morbidity and mortality (TRM), because of its own pathology and that associated with the immunosuppressive therapies generally used for its treatment. It is also a risk factor for the development of chronic GVHD.
Corticosteroids are the main first-line therapy for aGVHD, however these are associated with a response in less than 50 % of patients,4 and some patients who initially respond subsequently relapse.5 Response after short-term treatment with prednisolone (five days or 28 days) was significantly associated with a lower TRM than non-response.6,7 New therapies are therefore being investigated that can quickly and effectively treat aGVHD, and prevent its relapse. One such therapy is extracorporeal photopheresis (ECP).
ECP is a therapy that was originally developed over 25 years ago to treat cutaneous T-cell lymphoma.8 Since that time, its use has been investigated and shown promising efficacy in many other disorders with an auto- or alloimmune aetiology, including GVHD, systemic sclerosis, prevention and treatment of rejection in solid organ transplantation and Crohn’s disease.9 The treatment has three stages: first leukapheresis, following which the separated white blood cells are photoactivated with 8-methoxypsoralen and exposure to ultraviolet-A light, then return of the activated white blood cells to the patient. ECP has been found to be generally well tolerated, with a good safety profile, in patients with a variety of diseases.10 This paper reviews studies with ECP in patients with aGVHD.
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