Birgitta Versluijs

141 Outcome predictors in Allo-LS in children Twenty-four patients (45%) were noted as poor responders to first line therapy, after a median of 19 days (range 3-260), see Figure 1. In 13 (54%) of these patients, second line therapy was given, without success: 9 received fludarabine 30 mg/m2 every 3 weeks, but in earlier years, especially when there were signs of GVHD in other organs, daclizu- mab, infliximab, basiliximab, etanercept or Mesenchymal Stroma Cells were given. Of the ‘poor responders’ to MP pulse therapy 1 is alive after lung transplantation (5.3 years post HCT), and 2 are alive with end-stage lung disease requiring either intermittent chronic ventilator support or oxygen therapy. Only 1 child is alive without Allo-LS (good response on second line treatment). Twenty children died of progressive lung disease, with secondary fungal infection (2), viral reactivation (3: EBV, Adenovirus) or severe gut GvHD (2) contributing to death in seven cases. In the group of 30 ‘good responders’ 8 (26%) children died. There were no pulmonary deaths, 2 children died from infection (1 from systemic candida, 1 from invasive fungal infection). During prolonged MP-pulse therapy most children had important side effects like weight gain, osteoporosis, steroid induced diabetes mellitus and hypertension. FIGURE 1. Flow chart on response to therapy in 53 Allo-LS patients. Allo-LS, Alloimmune mediated lung syndrome 53 patients with Allo-LS 24 poor response to therapy 29 good response to therapy 2 died, infection 3 died, GVHD/multiorgan failure 3 died, primary disease 21 alive and well 13 salvage therapy  11 died, progressive  Allo-LS  1 end-stage lung  disease  1 alive without  lung disease 11 no salvage therapy  9 died, progressive  Allo-LS  1 lung transplan-  tation  1 end stage lung  disease 8