Treatment of children with drug-resistant Graft-Versus-Host-Disease by extracorporeal photopheresis

Abstract

PURPOSE/OBJECTIVE: We reviewed the clinical experience of performing extracorporeal photopheresis (ECP) for the treatment of children with drug-resistant graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation. MATERIALS AND METHODS: This is a retrospective review of children who received ECP at Queen Mary Hospital in Hong Kong since 2011. RESULTS: We performed ECP 79 times (discontinuous flow 16 times and continuous flow 63 times) in 3 children (body weight 26 to 35 kg) who suffered from severe extensive sclerodermatous chronic GVHD with pulmonary fibrosis (patient 1, beta-thalassemia major girl, matched unrelated stem cell transplant), steroid refractory grade 4 acute gut and liver GVHD (patient 2, AML boy, 5/6 HLA-matched unrelated cord blood transplant), and progressive bronchiolitis obliterans (patient 3, AML boy, matched sibling bone marrow transplant) respectively. Blood priming and prior hypertransfusions 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 reduction of the 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. CONCLUSIONS: ECP was well tolerated in these underweight patients by using the continuous flow photopheresis system. Technical problems are common and require trained and experienced nurses to perform the procedures. It seems to be an efficacious and safe alternative therapy for drug-resistant severe acute or chronic GVHD.