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Extracorporeal photopheresis for treatment of adults and children with acute GVHD: UK consensus statement and review of published literature

Bone Marrow Transplantation volume 49, pages 1251–1258 (2014)Cite this article

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Abstract

Extracorporeal photopheresis (ECP) has been used for over 20 years to treat acute GVHD (aGVHD) and chronic GVHD. Evidence on the efficacy of response in aGVHD has continued to accrue and data suggest that there is a good response and prolonged survival in both children and adults with grade II–IV aGVHD. Unlike chronic GVHD where treatment schedules are typically one or two times monthly for 12–18 months, patients with aGVHD respond rapidly to an intense weekly treatment schedule for 8 weeks, typically allowing steroids to be discontinued without flare-ups of aGVHD. Maintenance ECP therapy is generally not required. Many centres across Europe and United States treat aGVHD with ECP as second-line therapy and responses are excellent in a subset of patients. Unlike other second-line therapies, ECP is not immunosuppressive and has no reported drug interactions. Importantly, ECP does not have a negative impact on the graft-versus-malignancy effect of the transplant. This statement aims to select those patients most likely to respond to treatment and summarises treatment and monitoring schedules for the management of aGVHD in adult and paediatric patients to ensure the correct patients are treated with the optimal protocol for efficacy.

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References

  1. Edelson R, Berger C, Gasparro F, Jegasothy B, Heald P, Wintroub B et al. Treatment of cutaneous T-cell lymphoma by extracorporeal photochemotherapy. Preliminary results. New Engl J Med 1987; 316: 297–303.

    Article  CAS  PubMed  Google Scholar 

  2. Scarisbrick JJ, Taylor P, Holtick U, Makar Y, Douglas K, Berlin G et al. Photopheresis Expert Group UK. 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.

    Article  CAS  PubMed  Google Scholar 

  3. Martin PJ, Rizzo JD, Wingard JR, Ballen K, Curtin PT, Cutler C et al. First- and second-line systemic treatment of acute graft-versus-host disease: recommendations of the American Society of Blood and Marrow Transplantation. Biol. Blood Marrow Transplant 2012; 18: 1150–1163.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Jagasia M, Arora M, Flowers ME, Chao NJ, McCarthy PL, Cutler CS et al. Risk factors for acute GVHD and survival after hematopoietic cell transplantation. Blood 2012; 119: 296–307.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Couriel D, Saliba R, Hicks K, Ippoliti C, de Lima M, Hosing C et al. Tumor necrosis factor-alpha blockade for the treatment of aGvHD. Blood 2004; 104: 649–654.

    Article  CAS  PubMed  Google Scholar 

  6. Glucksberg H, Storb R, Fefer A, Buckner CD, Neiman PE, Clift RA et al. Clinical manifestations of graft-versus-host disease in human recipients of marrow from HL-A-matched sibling donors. Transplantation 1974; 18: 295–304.

    Article  CAS  PubMed  Google Scholar 

  7. Gratwohl A, Hermans J, Apperley J, Arcese W, Bacigalupo A, Bandini G et al. Acute graft-versus-host disease: grade and outcome in patients with chronic myelogenous leukemia. Working Party Chronic Leukemia of the European Group for Blood and Marrow Transplantation. Blood 1995; 86: 813–818.

    CAS  PubMed  Google Scholar 

  8. Dignan FL, Clark A, Amrolia P, Cornish J, Jackson G, Mahendra P et al. Diagnosis and management of acute graft-versus-host disease. Br J Haematol 2012; 158: 30–45.

    Article  CAS  PubMed  Google Scholar 

  9. MacMillan ML, Weisdorf DJ, Wagner JE, DeFor TE, Burns LJ, Ramsay NK et al. Response of 443 patients to steroids as primary therapy for acute graft-versus-host disease: comparison of grading systems. Biol Blood Marrow Transplant 2002; 8: 387–394.

    Article  CAS  PubMed  Google Scholar 

  10. Van Lint MT, Uderzo C, Locasciulli A, Majolino I, Scimé R, Locatelli F et al. Early treatment of acute graft-versus-host disease with high- or low-dose 6-methylprednisolone: a multicenter randomized trial from the Italian Group for Bone Marrow Transplantation. Blood 1998; 92: 2288–2293.

    CAS  PubMed  Google Scholar 

  11. Greinix HT, Volc-Platzer B, Rabitsch W, Gmeinhart B, Guevara-Pineda C, Kalhs P et al. Successful use of extracorporeal photochemotherapy in the treatment of severe acute and chronic graft-versus-host disease. Blood 1998; 92: 3098–3104.

    CAS  PubMed  Google Scholar 

  12. Miller JL, Goodman SA, Stricklin GP, Lloyd EK . Extracorporeal Photochemotherapy in the Treatment of Graft-versus-Host Disease. IBMTR/ABMTR: Keystone, CO, USA, 1998.

    Google Scholar 

  13. Smith EP, Sniecinski I, Dagis AC, Parker PM, Snyder DS, Stein AS et al. Extracorporeal photochemotherapy for treatment of drug-resistant graft-vs-host disease. Biol Blood Marrow Transplant 1998; 4: 27–37.

    Article  CAS  PubMed  Google Scholar 

  14. Girardi M, McNiff JM, Heald PW . Extracorporeal photochemotherapy in human and murine graft-versus-host disease. J Dermatol Sci 1999; 19: 106–113.

    Article  CAS  PubMed  Google Scholar 

  15. Greinix HT, Volc-Platzer B, Kalhs P, Fischer G, Rosenmayr A, Keil F et al. Extracorporeal photochemotherapy in the treatment of severe steroid-refractory acute graft-versus-host disease: a pilot study. Blood 2000; 96: 2426–2431.

    CAS  PubMed  Google Scholar 

  16. Salvaneschi L, Perotti C, Zecca M, Bernuzzi S, Viarengo G, Giorgiani G et al. Extracorporeal photochemotherapy for treatment of acute and chronic GVHD in childhood. Transfusion 2001; 41: 1299–1305.

    Article  CAS  PubMed  Google Scholar 

  17. Dall'Amico R, Messina C . Extracorporeal photochemotherapy for the treatment of graft-versus-host disease. Ther Apher 2002; 6: 296–304.

    Article  CAS  PubMed  Google Scholar 

  18. Greinix HT, Knobler RM, Worel N, Schneider B, Schneeberger A . The effect of intensified extracorporeal photochemotherapy on long-term survival in patients with severe acute graft-versus-host disease. Haematologica 2006; 91: 405–408.

    PubMed  Google Scholar 

  19. Greinix HT, Worel N, Knobler R . Role of extracorporeal photopheresis (ECP) in treatment of steroid-refractory acute graft-versus-host disease. Biol Blood Marrow Transplant 2010; 16: 1747–1748; author reply 1749.

    Article  PubMed  Google Scholar 

  20. Garban F, Drillat P, Makowski C, Jacob MC, Richard MJ, Favrot M et al. Extracorporeal chemophototherapy for the treatment of graft-versus-host disease: hematologic consequences of short-term, intensive courses. Haematologica 2005; 90: 1096–1101.

    PubMed  Google Scholar 

  21. Perfetti P, Carlier P, Strada P, Gualandi F, Occhini D, Van Lint MT et al. Extracorporeal photopheresis for the treatment of steroid refractory aGvHD. Bone Marrow Transplant 2008; 42: 609–617.

    Article  CAS  PubMed  Google Scholar 

  22. Besnier DP, Chabannes D, Mahé B, Mussini JM, Baranger TA, Muller JY et al. Treatment of graft-versus-host disease by extracorporeal photochemotherapy: a pilot study. Transplantation 1997; 64: 49–54.

    Article  CAS  PubMed  Google Scholar 

  23. Looks A, Fuchs D, Rulke D . Successful treatment of acute graft-versus-host disease after bone marrow transplantation in a 16-year-old girl with extracorporeal photopheresis. Onkologie 1997; 20: 340–342.

    Google Scholar 

  24. Richter HI, Stege H, Ruzicka T, Soehngen D, Heyll A, Krutmann J . Extracorporeal photopheresis in the treatment of acute graft-versus-host disease. J Am Acad Dermatol 1997; 36: 787–789.

    Article  CAS  PubMed  Google Scholar 

  25. Das-Gupta E, Greinix, Jacobs R, Zhou L, Savani BN, Engelhard BG et al. Extracorporeal photopheresis as second-line treatment for acute graft-versus-host disease: Impact on six month freedom from treatment failure. (in press).

  26. Jagasia M, Greinix H, Robin M, Das-Gupta E, Jacobs R, Savani BN et al. Extracorporeal photopheresis versus anticytokine therapy as a second-line treatment for steroid-refractory acute GVHD: a multicenter comparative analysis. Biol Blood Marrow Transplant 2013; 19: 1129–1133.

    Article  CAS  PubMed  Google Scholar 

  27. Messina C, Locatelli F, Lanino E, Uderzo C, Zacchello G, Cesaro S et al. Extracorporeal photochemotherapy for paediatric patients with graft-versus-host disease after haematopoietic stem cell transplantation. Br J Haematol 2003; 122: 118–127.

    Article  CAS  PubMed  Google Scholar 

  28. Berger M, Pessolano R, Albiani R, Asaftei S, Barat V, Carraro F et al. Extracorporeal photopheresis for steroid resistant graft versus host disease in pediatric patients: a pilot single institution report. J Pediatr Hematol Oncol 2007; 29: 678–687.

    Article  PubMed  Google Scholar 

  29. González Vicent M, Ramirez M, Sevilla J, Abad L, Díaz MA . Analysis of clinical outcome and survival in pediatric patients undergoing extracorporeal photopheresis for the treatment of steroid-refractory GVHD. J Pediatr Hematol Oncol 2010; 32: 589–593.

    Article  PubMed  Google Scholar 

  30. Kanold J, Merlin E, Halle P, Paillard C, Marabelle A, Rapatel C et al. Photopheresis in pediatric graft-versus-host disease after allogeneic marrow transplantation: clinical practice guidelines based on field experience and review of the literature. Transfusion 2007; 47: 2276–2289.

    Article  PubMed  Google Scholar 

  31. Calore E, Calò A, Tridello G, Cesaro S, Pillon M, Varotto S et al. Extracorporeal photochemotherapy may improve outcome in children with acute GVHD. Bone Marrow Transplant 2008; 42: 421–425.

    Article  CAS  PubMed  Google Scholar 

  32. Perotti C, Del Fante C, Tinelli C, Viarengo G, Scudeller L, Zecca M et al. Extracorporeal photochemotherapy in graft-versus-host disease: a longitudinal study on factors influencing the response and survival in pediatric patients. Transfusion 2010; 50: 1359–1369.

    Article  PubMed  Google Scholar 

  33. Szczepiorkowski ZM, Winters JL, Bandarenko N, Kim HC, Linenberger ML, Marques MB et al. Guidelines on the use of therapeutic apheresis in clinical practice—evidence-based approach from the Apheresis Applications Committee of the American Society for Apheresis. J Clin Apher 2010; 25: 83–177.

    Article  PubMed  Google Scholar 

  34. Szczepiorkowski ZM, Bandarenko N, Kim HC, Linenberger ML, Marques MB, Sarode R et al. Guidelines on the use of therapeutic apheresis in clinical practice: evidence-based approach from the Apheresis Applications Committee of the American Society for Apheresis. J Clin Apher 2007; 22: 106–175.

    Article  PubMed  Google Scholar 

  35. Knobler R, Berlin G, Calzavara-Pinton P, Greinix H, Jaksch P, Laroche L et al. Guidelines on the use of extracorporeal photopheresis. J Eur Acad Dermatol Venereol 2014; 1: 1–37.

    Article  Google Scholar 

  36. McKenna KE, Whittaker S, Rhodes LE, Taylor P, Lloyd J, Ibbotson S et al. Evidence-based practice of photopheresis 1987–2001: a report of a workshop of the British Photodermatology Group and the U.K. Skin Lymphoma Group. Br J Dermatol 2006; 154: 7–20.

    Article  CAS  PubMed  Google Scholar 

  37. Pierelli L, Perseghin P, Marchetti M, Messina C, Perotti C, Mazzoni A et al. Extracorporeal photopheresis for the treatment of acute and chronic graft-versus-host disease in adults and children: best practice recommendations from an Italian Society of Hemapheresis and Cell Manipulation (SIdEM) and Italian Group for Bone Marrow Transplantation (GITMO) consensus process. Transfusion 2013; 53: 2340–2352.

    PubMed  Google Scholar 

  38. Apsner R, Uenver B, Sunder-Plassmann G, Knobler RM . Regional anticoagulation with acid citrate dextrose-A for extracorporeal photoimmunochemotherapy. Vox Sang 2002; 83: 222–226.

    Article  CAS  PubMed  Google Scholar 

  39. Nedelcu E, Ziman A, Fernando LP, Cook K, Bumerts P, Schiller G . Exclusive use of acid citrate dextrose for anticoagulation during extracorporeal photopheresis in patients with contraindications to heparin: an effective protocol. J Clin Apher 2008; 23: 66–73.

    Article  PubMed  Google Scholar 

  40. Sniecinski I, Smith B, Parker PM, Dagis A . Extracorporeal photochemotherapy for the treatment of graft-versus-host disease. J Clin Apher 1995; 10: 51.

    Google Scholar 

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Acknowledgements

We are grateful to the members of UK Photopheresis Group in their support in preparing these guidelines.

Author information

Author notes

  1. E Das-Gupta, F Dignan, B Shaw, K Raj, R Malladi, A Gennery and D Bonney: Member of Photopheresis Expert Group of the UK Photopheresis Society.

  2. P Taylor: Chairperson.

  3. J Scarisbrick: Secretary.

Authors and Affiliations

  1. Centre for Clinical Haematology, Nottingham University Hospitals NSH Trust, Nottingham, UK

    E Das-Gupta

  2. Central Manchester University Hospitals, Manchester, UK

    F Dignan

  3. Royal Marsden Hospital, London, UK

    B Shaw

  4. King’s College Hospital, London, UK

    K Raj

  5. University Hospital Birmingham NHS Trust, Birmingham, UK

    R Malladi & J Scarisbrick

  6. Great North Children’s Hospital, Newcastle-Upon-Tyne, UK

    A Gennery

  7. Royal Manchester Children’s Hospital, Manchester, UK

    D Bonney

  8. Rotherham Foundation NHS Trust, Rotherham, South Yorkshire, UK

    P Taylor

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Corresponding author

Correspondence to E Das-Gupta.

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Competing interests

FLD, JJS and BES have received research funding, honoraria and speaker’s fees from Therakos. EDG has received honoraria and speaker’s fees from Therakos. PCT has received speaker fees from Therakos. KR has received sponsorship for attendance at conferences from Therakos. None of the other authors have declared any conflicts of interest.

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Das-Gupta, E., Dignan, F., Shaw, B. et al. Extracorporeal photopheresis for treatment of adults and children with acute GVHD: UK consensus statement and review of published literature. Bone Marrow Transplant 49, 1251–1258 (2014). https://doi.org/10.1038/bmt.2014.106

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