Background: End-stage lung disease is a rare complication of treatment for hematologic and solid tumors in children. When present, it is generally progressive, resulting in the patient being cured of cancer only to die of respiratory failure. Lung transplantation is believed by some to be of overly high risk in this population because of the pre-existing malignancy as well as the presumed compromised immune status.
Methods: Six children (Group 1), aged 5 to 17 years (mean 12.4 years), underwent lung transplantation at our institution because of either pulmonary fibrosis or bronchiolitis obliterans following treatment for acute leukemia (n = 5) or medulloblastoma (n = 1). All patients received chemotherapy, radiation and bone marrow transplantation in the course of treatment for their malignancies. The average forced expiratory volume in 1 second (FEV(1)) was 16% of the predicted value and forced vital capacity (FVC) was 28% of predicted. These results were compared with a similar group of 13 patients undergoing lung transplantation at our institution during the same time interval (Group 2).
Results: There were 2 deaths in Group 1, 1 early and 1 late, for an overall survival of 67% at a mean follow-up of 4.02 years. There were no early and 7 late deaths in Group 2 for an overall survival of 46% at a mean follow-up of 4.8 years. The acute rejection rate in the first post-transplant year was 0.2 episode/patient in Group 1 and 1.8 episode/patient in Group 2 (p <0.01). No patient in Group 1 has developed post-transplant lymphoproliferative disease or a relapse of their primary malignancy. Two patients in Group 1 developed unusual infections-Aspergillus and Mycobacterium chelonae. No such infections occurred in Group 2.
Conclusions: Although this represents a small series, we believe that patients with respiratory failure following treatment of a prior malignancy are suitable candidates for lung transplantation. Although they may have some relative protection from acute rejection episodes by virtue of an already compromised immunologic status while receiving standard immunosuppression, an increased propensity for opportunistic infection may exist.