RT Journal Article
SR Electronic
T1 An Evidence-Based Guideline Improves Outcomes for Patients With Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1042
OP 1051
DO 10.3899/jrheum.211219
VO 49
IS 9
A1 Maria L. Taylor
A1 Kacie J. Hoyt
A1 Joseph Han
A1 Leslie Benson
A1 Siobhan Case
A1 Mia T. Chandler
A1 Margaret H. Chang
A1 Craig Platt
A1 Ezra M. Cohen
A1 Megan Day-Lewis
A1 Fatma Dedeoglu
A1 Mark Gorman
A1 Jonathan S. Hausmann
A1 Erin Janssen
A1 Pui Y. Lee
A1 Jeffrey Lo
A1 Gregory P. Priebe
A1 Mindy S. Lo
A1 Esra Meidan
A1 Peter A. Nigrovic
A1 Jordan E. Roberts
A1 Mary Beth F. Son
A1 Robert P. Sundel
A1 Maria Alfieri
A1 Jenny Chan Yeun
A1 Damilola M. Shobiye
A1 Barbara Degar
A1 Joyce C. Chang
A1 Olha Halyabar
A1 Melissa M. Hazen
A1 Lauren A. Henderson
YR 2022
UL http://www.jrheum.org/content/49/9/1042.abstract
AB Objective To compare clinical outcomes in children with hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) who were managed before and after implementation of an evidence-based guideline (EBG).Methods A management algorithm for MAS-HLH was developed at our institution based on literature review, expert opinion, and consensus building across multiple pediatric subspecialties. An electronic medical record search retrospectively identified hospitalized patients with MAS-HLH in the pre-EBG (October 15, 2015, to December 4, 2017) and post-EBG (January 1, 2018, to January 21, 2020) time periods. Predetermined outcome metrics were evaluated in the 2 cohorts.Results After the EBG launch, 57 children were identified by house staff as potential patients with MAS-HLH, and rheumatology was consulted for management. Ultimately, 17 patients were diagnosed with MAS-HLH by the treating team. Of these, 59% met HLH 2004 criteria, and 94% met 2016 classification criteria for MAS complicating systemic juvenile idiopathic arthritis. There was a statistically significant reduction in mortality from 50% before implementation of the EBG to 6% in the post-EBG cohort (P = 0.02). There was a significant improvement in time to 50% reduction in C-reactive protein level in the post-EBG vs pre-EBG cohorts (log-rank P < 0.01). There were trends toward faster time to MAS-HLH diagnosis, faster initiation of immunosuppressive therapy, shorter length of hospital stay, and more rapid normalization of MAS-HLH–related biomarkers in the patients post-EBG.Conclusion While the observed improvements may be partially attributed to advances in treatment of MAS-HLH that have accumulated over time, this analysis also suggests that a multidisciplinary treatment pathway for MAS-HLH contributed meaningfully to favorable patient outcomes.