Extracorporeal photopheresis: an effective treatment for pulmonary graft versus host disease

Introduction: Pulmonary graft versus host disease (GvHD) is a rare but severe complication post bone marrow transplant (BMT). Corticosteroids and immunosuppressive drugs have limited success in managing GvHD. We present a case of a 49 year-old lady whose lung GvHD responded well to ECP (extracorporeal photopheresis). Case: The patient presented to chest clinic in 2015 with a history of sibling allogenic BMT in 1982 for aplastic anaemia. She had mucocutaneous GvHD, managed with ciclosporin 50mg BD and mycophenolate 750mg BD. CT chest showed bronchiolitis obliterans, consistent with lung GvHD. Mycophenolate was increased to 1g BD along with an inhaled corticosteroid and long acting bronchodilator, montelukast and erythromycin prophylaxis. Aften initial improvement, her lung function declined (table). The option of escalating immunosuppresion was limited by recurrent skin cancers. She commenced ECP in early 2018 which improved her lung function and her cutaneous GvHD. Her FEV1 and FVC improved by 15% and 22% respectively (table). Discussion: Treatment options for pulmonary GvHD include steroids, azathioprine, mycophenolate, anti-TNFα, ciclosporin and rapamycin (Hildebrandt et al. BMT. 2011. 46: 1283–95). ECP is recommended for resistant cases but is not easily accessibile. We hope this case will improve awareness amongst respiratory physicians of ECP as an effective treatment option in severe pulmonary GvHD.