The use of photopheresis or Extra Corporeal Perfusion (ECP) as a treatment of graft-versus-host disease (GVHD) after a prior allogeneic stem cell transplant is based on the fact that GVHD is similar to autoimmune disease in that it too is an immunologically mediated disease. Chronic GVHD typically presents with more diverse symptomatology resembling autoimmune diseases such as progressive systemic sclerosis, systemic lupus erythematosus, or rheumatoid arthritis. It may affect the mouth, eyes, respiratory tract, musculoskeletal system, peripheral nerves, as well as the skin, liver, or gut – the usual sites of acute GVHD.
A 2001 BlueCross BlueShield Association Technology Evaluation Center (TEC) Assessment, which offered the following observations and conclusions:
For acute GVHD or chronic GVHD in previously untreated patients, or in those responding to conventional therapy, there were no studies that met selection criteria and reported results of extracorporeal photopheresis, alone or in combination with other therapies. Therefore, it was not possible to draw conclusions concerning the effects of this therapy on health outcomes in previously untreated or responsive patients.
In acute GVHD, a phase II study by Greinix et al. involving 38 patients reported complete remission in 86%, 55%, and 30% of patients with grades 2, 3, and 4 agvhd respectively. The best results were obtained in 82%, 61%, and 61% of patients with skin, liver, and gut agvhd respectively.
Studies focusing on patients with chronic GVHD unresponsive to other therapies reported resolution or marked improvement of lesions in about 50% of patients. The one phase II study by Flowers et al. reported on a multicentre prospective phase II randomized study in 23 transplant centres in North and South America, Europe, and Australia. The 95 enrolled patients were randomized either to ECP plus standard therapy or to standard therapy alone. The conclusion reached was that ECP had a steroid-sparing effect in the treatment of chronic GVHD.
Three studies reported outcomes for 38 patients with acute GVHD that was refractory to standard treatment with steroids and other immunosuppressive drugs. Patients with Grade IV disease were generally unresponsive to photopheresis. While a single study of 21 patients reported responses in a majority of patients with Grade III disease, the small number of patients in this study was not sufficient to permit conclusions concerning the outcomes of photopheresis for treatment-refractory acute GVHD. SInce then there ahd been a nubmer of otehr reprots but no studies. A 2008 workshop convcded: “The evidence for the efficacy of ECP has been appraised by a combined British Photodermatology Group and U.K. Skin Lymphoma Group workshop on the basis of evidence published up to the end of 2001 and on the consensus of best practice. There is fair evidence for the use of ECP in erythrodermic CTCL and steroid-refractory GVHD, but randomized controlled studies are needed.” National Institute for Health and Clinical Excellence in the UK endorsed the use of ECP for CTCL and because of the complexity of treatment supported its use in specialized centres and also suggested the need for expansion of this service.
A 2012 guideline(Dinghan et al) recommends it as second line treatment.
Dignan FL, Amrolia P, Clark A, Cornish J, Jackson G, Mahendra P, Scarisbrick JJ, Taylor PC, Shaw BE, Potter MN, on behalf of the Haemato-oncology Task Force of the British Committee [trunc]. Diagnosis and management of chronic graft-versus-host disease. Br J Haematol 2012 Jul;158(1):46-61. [125 references]
Greinix HT, Knobler RM, Worel N. The effect of intensified extracorporeal photochemotherapy on long-term survival in patients with severe acute graft-versus-host disease. Haematologica. 2006;91:405–8.
Scarisbrick JJ, Taylor P, Holtick U, et al. U.K. consensus statement on the use of extracorporeal photopheresis for treatment of cutaneous T-cell lymphoma and chronic graft-versus-host disease. Br J Dermatol. 2008;158:659–678.
J. Klassen, The role of photopheresis in the treatment of graft-versus-host disease Curr Oncol. 2010 April; 17(2): 55–58.
Halle P, Paillard C, D’Incan M et al. Successful extracorporeal photochemotherapy for chronic graft-versus-host disease in pediatric patients. J Hematother Stem Cell Res 2002;11(3):501-12
Salvaneschi L, Perotti C, Zecca M et al. Extracorporeal photochemotherapy for treatment of acute and chronic GVHD in childhood. Transfusion 2001;41(10):1299-305
Flowers ME, Apperley JF, van Besien K. A multi-center prospective phase 2 randomized study of extracorporeal photopheresis for treatment of chronic graft-versus-host disease. Blood. 2008;112:2667–74. [Erratum in: Blood 2009;113:4478]
71 year-old man s/p reduced intensisty allogeneic transplant from sister after AML diagnosed and induced in May 2010. He developed Graft vs Host Disease and was treated with tacrolimus but now received photopheresis.