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 p...

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Veröffentlicht in:Pediatric rheumatology online journal Jg. 17; H. 1; S. 7 - 12
Hauptverfasser: 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.
Format: Journal Article
Sprache:Englisch
Veröffentlicht: London BioMed Central 14.02.2019
BioMed Central Ltd
Springer Nature B.V
BMC
Schlagworte:
Algorithms
Antigens
Arthritis
Care and treatment
Chemotherapy
Collaboration
Consensus
Cytokine storm
Cytokines
Cytokines - blood
Diagnosis
Diagnosis, Differential
Evidence-based guideline
Evidence-Based Medicine - methods
Ferritin
Fever
Health aspects
Hemophagocytic lymphohistiocytosis
Hemophagocytic lymphohistiocytosis (HLH)
Humans
Immunoglobulins
Infections
Integrative medicine
Lymphatic diseases
Lymphohistiocytosis, Hemophagocytic - diagnosis
Lymphohistiocytosis, Hemophagocytic - therapy
Macrophage activation syndrome (MAS)
Macrophage Activation Syndrome - diagnosis
Macrophage Activation Syndrome - therapy
Macrophages
Medical diagnosis
Medicine
Medicine & Public Health
Methods
Mortality
Mutation
Patients
Pediatric research
Pediatrics
Practice Guidelines as Topic
Quality control
Quality Improvement
Quality improvement research
Research Article
Rheumatic diseases
Rheumatoid arthritis
Rheumatology
Sepsis
Strategic planning (Business)
Hemophagocytic lymphohistiocytosis (HLH)
Macrophage activation syndrome (MAS)
Evidence-based guideline
Quality improvement research
ISSN:1546-0096, 1546-0096
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Zusammenfassung: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.
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ISSN:1546-0096
1546-0096
DOI:10.1186/s12969-019-0309-6