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Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) were historically thought to be distinct entities, often managed in isolation. In fact, these conditions are closely related. A collaborative approach, which incorporates expertise from subspecialties that...

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Autores principales: Halyabar, Olha, Chang, Margaret H., Schoettler, Michelle L., Schwartz, Marc A., Baris, Ezgi H., Benson, Leslie A., Biggs, Catherine M., Gorman, Mark, Lehmann, Leslie, Lo, Mindy S., Nigrovic, Peter A., Platt, Craig D., Priebe, Gregory P., Rowe, Jared, Sundel, Robert P., Surana, Neeraj K., Weinacht, Katja G., Mann, Alison, Yuen, Jenny Chan, Meleedy-Rey, Patricia, Starmer, Amy, Banerjee, Taruna, Dedeoglu, Fatma, Degar, Barbara A., Hazen, Melissa M., Henderson, Lauren A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6376762/
https://www.ncbi.nlm.nih.gov/pubmed/30764840
http://dx.doi.org/10.1186/s12969-019-0309-6
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author Halyabar, Olha
Chang, Margaret H.
Schoettler, Michelle L.
Schwartz, Marc A.
Baris, Ezgi H.
Benson, Leslie A.
Biggs, Catherine M.
Gorman, Mark
Lehmann, Leslie
Lo, Mindy S.
Nigrovic, Peter A.
Platt, Craig D.
Priebe, Gregory P.
Rowe, Jared
Sundel, Robert P.
Surana, Neeraj K.
Weinacht, Katja G.
Mann, Alison
Yuen, Jenny Chan
Meleedy-Rey, Patricia
Starmer, Amy
Banerjee, Taruna
Dedeoglu, Fatma
Degar, Barbara A.
Hazen, Melissa M.
Henderson, Lauren A.
author_facet Halyabar, Olha
Chang, Margaret H.
Schoettler, Michelle L.
Schwartz, Marc A.
Baris, Ezgi H.
Benson, Leslie A.
Biggs, Catherine M.
Gorman, Mark
Lehmann, Leslie
Lo, Mindy S.
Nigrovic, Peter A.
Platt, Craig D.
Priebe, Gregory P.
Rowe, Jared
Sundel, Robert P.
Surana, Neeraj K.
Weinacht, Katja G.
Mann, Alison
Yuen, Jenny Chan
Meleedy-Rey, Patricia
Starmer, Amy
Banerjee, Taruna
Dedeoglu, Fatma
Degar, Barbara A.
Hazen, Melissa M.
Henderson, Lauren A.
author_sort Halyabar, Olha
collection PubMed
description BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) were historically thought to be distinct entities, often managed in isolation. In fact, these conditions are closely related. A collaborative approach, which incorporates expertise from subspecialties that previously treated HLH/MAS independently, is needed. We leveraged quality improvement (QI) techniques in the form of an Evidence-Based Guideline (EBG) to build consensus across disciplines on the diagnosis and treatment of HLH/MAS. METHODS: A multidisciplinary work group was convened that met monthly to develop the HLH/MAS EBG. Literature review and expert opinion were used to develop a management strategy for HLH/MAS. The EBG was implemented, and quality metrics were selected to monitor outcomes. RESULTS: An HLH/MAS clinical team was formed with representatives from subspecialties involved in the care of patients with HLH/MAS. Broad entry criteria for the HLH/MAS EBG were established and included fever and ferritin ≥500 ng/mL. The rheumatology team was identified as the “gate-keeper,” charged with overseeing the diagnostic evaluation recommended in the EBG. First-line medications were recommended based on the acuity of illness and risk of concurrent infection. Quality metrics to be tracked prospectively based on time to initiation of treatment and clinical response were selected. CONCLUSION: HLH/MAS are increasingly considered to be a spectrum of related conditions, and joint management across subspecialties could improve patient outcomes. Our experience in creating a multidisciplinary approach to HLH/MAS management can serve as a model for care at other institutions. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12969-019-0309-6) contains supplementary material, which is available to authorized users.
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spelling pubmed-63767622019-02-27 Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome Halyabar, Olha Chang, Margaret H. Schoettler, Michelle L. Schwartz, Marc A. Baris, Ezgi H. Benson, Leslie A. Biggs, Catherine M. Gorman, Mark Lehmann, Leslie Lo, Mindy S. Nigrovic, Peter A. Platt, Craig D. Priebe, Gregory P. Rowe, Jared Sundel, Robert P. Surana, Neeraj K. Weinacht, Katja G. Mann, Alison Yuen, Jenny Chan Meleedy-Rey, Patricia Starmer, Amy Banerjee, Taruna Dedeoglu, Fatma Degar, Barbara A. Hazen, Melissa M. Henderson, Lauren A. Pediatr Rheumatol Online J Research Article BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) were historically thought to be distinct entities, often managed in isolation. In fact, these conditions are closely related. A collaborative approach, which incorporates expertise from subspecialties that previously treated HLH/MAS independently, is needed. We leveraged quality improvement (QI) techniques in the form of an Evidence-Based Guideline (EBG) to build consensus across disciplines on the diagnosis and treatment of HLH/MAS. METHODS: A multidisciplinary work group was convened that met monthly to develop the HLH/MAS EBG. Literature review and expert opinion were used to develop a management strategy for HLH/MAS. The EBG was implemented, and quality metrics were selected to monitor outcomes. RESULTS: An HLH/MAS clinical team was formed with representatives from subspecialties involved in the care of patients with HLH/MAS. Broad entry criteria for the HLH/MAS EBG were established and included fever and ferritin ≥500 ng/mL. The rheumatology team was identified as the “gate-keeper,” charged with overseeing the diagnostic evaluation recommended in the EBG. First-line medications were recommended based on the acuity of illness and risk of concurrent infection. Quality metrics to be tracked prospectively based on time to initiation of treatment and clinical response were selected. CONCLUSION: HLH/MAS are increasingly considered to be a spectrum of related conditions, and joint management across subspecialties could improve patient outcomes. Our experience in creating a multidisciplinary approach to HLH/MAS management can serve as a model for care at other institutions. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12969-019-0309-6) contains supplementary material, which is available to authorized users. BioMed Central 2019-02-14 /pmc/articles/PMC6376762/ /pubmed/30764840 http://dx.doi.org/10.1186/s12969-019-0309-6 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research Article
Halyabar, Olha
Chang, Margaret H.
Schoettler, Michelle L.
Schwartz, Marc A.
Baris, Ezgi H.
Benson, Leslie A.
Biggs, Catherine M.
Gorman, Mark
Lehmann, Leslie
Lo, Mindy S.
Nigrovic, Peter A.
Platt, Craig D.
Priebe, Gregory P.
Rowe, Jared
Sundel, Robert P.
Surana, Neeraj K.
Weinacht, Katja G.
Mann, Alison
Yuen, Jenny Chan
Meleedy-Rey, Patricia
Starmer, Amy
Banerjee, Taruna
Dedeoglu, Fatma
Degar, Barbara A.
Hazen, Melissa M.
Henderson, Lauren A.
Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome
title Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome
title_full Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome
title_fullStr Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome
title_full_unstemmed Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome
title_short Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome
title_sort calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6376762/
https://www.ncbi.nlm.nih.gov/pubmed/30764840
http://dx.doi.org/10.1186/s12969-019-0309-6
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