Rohit P Nair, Michael C Mowrer, David Farbo, James A Thomas, Andrea Ontaneda, Saad Ghafoor, Renee M Potera, Lakshmi Raman, Saleh Bhar, New Collective Author
Hemophagocytic lymphohistiocytosis (HLH) is a rare condition causing severe systemic inflammatory activation, cytokine storm, and multiorgan dysfunction for which extracorporeal membrane oxygenation (ECMO) may be required. There is a paucity of data to guide clinicians in the care of these patients; we aimed to address this need. From the Hematology and Oncology Pediatric Patients on ECMO (HOPPE) database, 30 patients diagnosed with HLH were identified from 13 of the 16 total participating centers between 2009 and 2021. Of those 30 patients, 63% survived to ECMO decannulation, 43% to ICU discharge, and 37% to hospital discharge. Among survivors, there was a median of 44.9 days between decannulation and hospital discharge. Half of the ECMO mortality occurred within the first 10 days on ECMO. Patients who survived to hospital discharge also had evidence of functional limitation and significant multiorgan dysfunction. A total of 90% of patients diagnosed with HLH on ECMO survived to decannulation vs. 50% of patients diagnosed before. Survival has improved in this patient population compared to previous literature but is still lower than the overall pediatric ECMO population. Clinical suspicion for HLH in these critically ill patients should not exclude them from escalation of support, including ECMO.