Evidence map›Paper›PMID 35840156›Full record

ArticleThe Journal of rheumatology2022

An Evidence-Based Guideline Improves Outcomes for Patients With Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome.

Maria L Taylor, Kacie J Hoyt, Joseph Han, Leslie Benson, Siobhan Case, Mia T Chandler, Margaret H Chang, Craig Platt, Ezra M Cohen, Megan Day-Lewis and 21 more

Open access · greenAbstract read

PubMed Full text at PMC Publisher

In one paragraph

Article in The Journal of rheumatology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.

0numbers the graph read from it

0cells of the map it votes in

6citing papers in PubMed

2.4field-weighted citation impact, top 10% of its field

1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

6 citing papers in PubMed, 17 citations in OpenAlex.

Advancements in Diagnosis and Care for Immune Dysregulation and Inborn Errors of Immunity in Pediatric Rheumatology: Our 50-Year Journey.Arthritis care & research · 2026
Review
Identifying Hemophagocytic Lymphohistiocytosis and Describing Outcomes Using Computable Phenotypes: Retrospective Cohort Study.JMIR cancer · 2026
Article
Multidisciplinary approach to treating complex immune dysregulation disorders: an adaptive model for institutional implementation.Frontiers in immunology · 2025
Review
Treating and triggering hyperinflammation: tackling hemophagocytic lymphohistiocytosis and HLH-like syndromes in the pediatric cell therapy and critical care setting.Frontiers in oncology · 2025
Review
Hyperferritinemia Screening to Aid Identification and Differentiation of Patients with Hyperinflammatory Disorders.Journal of clinical immunology · 2024
Article
[Hemophagocytic lymphohistiocytosis and macrophage activation syndrome : A multidisciplinary challenge].Zeitschrift fur Rheumatologie · 2024
Review

4 · The record

Corrections and comments

Commented on by
Improving Outcomes for Patients With Hemophagocytic Lymphohistiocytosis.2023

5 · Who and what money

Authors and funding

31 authors at 4 institutions in 1 country.

Maria L TaylorM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.ORCID 0000-0002-5761-7365

Kacie J HoytK.J. Hoyt, MSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts, and Virginia Tech Carilion School of Medicine, Roanoke, Virginia.

Joseph HanJ. Han, BS, Icahn School of Medicine at Mount Sinai, New York, New York.ORCID 0000-0002-8563-6926

Leslie BensonL. Benson, MD, M. Gorman, MD, Division of Neurology, Boston Children's Hospital, Boston, Massachusetts.

Siobhan CaseS. Case, MD, M.H. Chang, MD, PhD, P.A. Nigrovic, MD, Division of Immunology, Boston Children's Hospital, and Division of Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Boston, Massachusetts.

Mia T ChandlerM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.ORCID 0000-0003-2223-0061

Margaret H ChangS. Case, MD, M.H. Chang, MD, PhD, P.A. Nigrovic, MD, Division of Immunology, Boston Children's Hospital, and Division of Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Boston, Massachusetts.

Craig PlattM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Ezra M CohenM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Megan Day-LewisM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Fatma DedeogluM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Mark GormanL. Benson, MD, M. Gorman, MD, Division of Neurology, Boston Children's Hospital, Boston, Massachusetts.

Jonathan S HausmannJ.S. Hausmann, MD, Division of Immunology, Boston Children's Hospital, and Division of Rheumatology and Clinical Immunology, Beth Israel Deaconess Medical Center, Boston, Massachusetts.

Erin JanssenM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Pui Y LeeM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Jeffrey LoM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Gregory P PriebeG.P. Priebe, MD, Department of Anesthesiology, Critical Care, and Pain Medicine, Boston Children's Hospital, Boston, Massachusetts.

Mindy S LoM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.ORCID 0000-0003-2328-3536

Esra MeidanM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Peter A NigrovicS. Case, MD, M.H. Chang, MD, PhD, P.A. Nigrovic, MD, Division of Immunology, Boston Children's Hospital, and Division of Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Boston, Massachusetts.

Jordan E RobertsM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Mary Beth F SonM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Robert P SundelM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Maria AlfieriM. Alfieri, MPH, J. Chan Yeun, MSPH, D.M. Shobiye, MPH, Department of Pediatric Quality Program, Boston Children's Hospital, Boston, Massachusetts.

Jenny Chan YeunM. Alfieri, MPH, J. Chan Yeun, MSPH, D.M. Shobiye, MPH, Department of Pediatric Quality Program, Boston Children's Hospital, Boston, Massachusetts.

Damilola M ShobiyeM. Alfieri, MPH, J. Chan Yeun, MSPH, D.M. Shobiye, MPH, Department of Pediatric Quality Program, Boston Children's Hospital, Boston, Massachusetts.ORCID 0000-0002-0816-6836

Barbara DegarB. Degar, MD, Department of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.

Joyce C ChangM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Olha HalyabarM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Melissa M HazenM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts.

Lauren A HendersonM.L. Taylor, BS, M.T. Chandler, MD, C. Platt, MD, PhD, E.M. Cohen, MD, M. Day-Lewis, RN, MSN, CPNP, F. Dedeoglu, MD, E. Janssen, MD, PhD, P.Y. Lee, MD, PhD, J. Lo, MD, M.S. Lo, MD, PhD, E. Meidan, MD, J.E. Roberts, MD, M.B.F. Son, MD, R.P. Sundel, MD, J.C. Chang, MD, MSCE, O. Halyabar, MD, M.M. Hazen, MD, L.A. Henderson, MD, MMSc, Division of Immunology, Boston Children's Hospital, Boston, Massachusetts; Lauren.Henderson@childrens.harvard.edu.ORCID 0000-0003-0242-4029

Boston Children's Hospital · USBrigham and Women's Hospital · USDana-Farber Cancer Institute · USIcahn School of Medicine at Mount Sinai · US

Funding

MOLECULAR BASIS OF ALLERGIC AND IMMUNOLOGIC DISEASET32AI007512 · NIAID · CHILDREN'S HOSPITAL BOSTON · PI Janet Chou, Peter A Nigrovic · 1996 to 2026

$13.1M

Joint Biology Consortium Resource-based CenterP30AR070253 · NIAMS · BRIGHAM AND WOMEN'S HOSPITAL · PI Peter A Nigrovic, Jeffrey Andrew Sparks · 2016 to 2026

$9.4M

The Child Heath Research CenterK12HD052896 · NICHD · BOSTON CHILDREN'S HOSPITAL · PI Wendy K Chung · 2007 to 2026

$7.9M

The Reprogramming of Regulatory T cells to a Th17 Phenotype in Systemic Juvenile Idiopathic ArthritisK08AR073339 · NIAMS · BOSTON CHILDREN'S HOSPITAL · PI HENDERSON, LAUREN A · 2018 to 2022

$840k

NIAID NIH HHS T32 AI007512NIAMS NIH HHS K08 AR073339NIAMS NIH HHS P30 AR070253NICHD NIH HHS K12 HD052896

6 · The paper itself

Abstract

objectiveTo 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).

methodsA 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.

resultsAfter 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 (

conclusionWhile 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.

Indexed as

Lymphohistiocytosis, HemophagocyticMacrophage Activation SyndromeBiomarkersChildC-Reactive ProteinHumansRetrospective StudiesBiomarkersC-Reactive Proteinhemophagocytic lymphohistiocytosismacrophage activation syndrome

Identifiers

PMID35840156

DOI10.3899/jrheum.211219

PMCPMC9588491

OpenAlexW4285590055

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