Treatment of children with Drugresistant Graft-Versus-Host-Disease by extra-corporeal photopheresis

Abstract

PURPOSE AND METHOD: We retrospectively reviewed the clinical experience of performing extracorporeal photopheresis (ECP) for the treatment of children with drugresistant graft-versus-host disease (GVHD) after allogeneic haematopoietic stem cell transplantation at Queen Mary Hospital in Hong Kong since 2011. RESULTS: We performed ECP 89 times (discontinuous flow 16 times and continuous flow 73 times) in 3 children (body weight 26 to 35 kg) who suffered from severe extensive sclerodermatous chronic GVHD with pulmonary fibrosis (patient 1, beta-thalassaemia major girl received matched unrelated stem cell transplant), steroid refractory grade 4 acute gut and liver GVHD (patient 2, AML boy received 5/6 HLA-matched unrelated cord blood transplant), and progressive bronchiolitis obliterans (patient 3, AML boy received matched sibling bone marrow transplant) respectively. Nurse is a key stakeholder in managing the technical and logistic parts of ECP program. The nurse's roles are discussed on developing the ECP program, setting operating standard, case management, problem encountered and staff training. The ECP procedures in small children are technically challenging and require special planning with attention to extracorporeal volume, inlet blood flow rate and trouble shooting. Blood priming and prior hyper-transfusions were required to minimize fluid shifts in these underweight patients. ECP was performed 1-3 times per week. The anticoagulant: blood ratio (heparin saline 20 units/ml) was adjusted according to the patients' hemostatic status between 8:1 to 12:1. Problems including machine failure possibly caused by poor inlet blood flow, blood clots or air in the circuits were encountered. Transient hypertension and cold sensation had been reported during discontinuous flow but not in continuous flow ECP. There were no adverse hemodynamic events noted. Patient 2 and 3 had significant improvement in their GVHD resulting in the reduction of immunosuppressive drugs. Patient 1 started ECP at a relatively late stage and she died of pulmonary aspergillosis and atypical mycobacterial infection, unrelated to the procedure. The nurse-led ECP program gained high satisfaction from staff and patients. CONCLUSION: ECP was well tolerated in these underweight patients by using the continuous flow photopheresis system. It seems to be an efficacious and safe alternative therapy for drugresistant severe acute or chronic GVHD. A nurse-led ECP program facilitates the success of technical and logistic parts of this new treatment modality.