Non-HLA Antibody-Associated Hyperacute Allograft Rejection Following Lung Transplantation.

BACKGROUND: Pediatric-onset pulmonary hypertension (PH) is a rare, progressive pulmonary vascular disease associated with high mortality. In cases unresponsive to pharmacological therapy with a severe cardiorespiratory phenotype, lung transplantation (LT) remains the final treatment option. Although LT for pediatric PH is considered a procedure with elevated risk, it has been successfully performed in this population. CASE PRESENTATION: A 3-year-old boy underwent uncomplicated LT for fulminant TBX4-associated PH and developed hyperacute allograft failure requiring plasmapheresis, augmentation of immunosuppression, and the use of a monoclonal complement inhibitor. Despite extensive treatment the patient died 46 days after LT. Retrospective analysis of the serum sample taken at time of transplant prior to reperfusion was positive for six non-histocompatibility antibodies: Sjogren syndrome antigen B, thyroglobulin, glutathione S-transferase Theta-1, colony stimulating factor 2, intracellular adhesion molecule 1, and interferon gamma that were not present in other pediatric thoracic transplant recipients. CONCLUSIONS: Hyperacute allograft rejection is extremely rare in LT recipients and its occurrence in this patient implies the need for further characterization of non-HLA antibody-associated LT rejection.