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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.

Abstract：

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.

journal_name

Pediatr Rheumatol Online J

journal_title

Pediatric rheumatology online journal

authors

Halyabar O,Chang MH,Schoettler ML,Schwartz MA,Baris EH,Benson LA,Biggs CM,Gorman M,Lehmann L,Lo MS,Nigrovic PA,Platt CD,Priebe GP,Rowe J,Sundel RP,Surana NK,Weinacht KG,Mann A,Yuen JC,Meleedy-Rey P,Starmer A,Ban

doi

10.1186/s12969-019-0309-6

subject

Has Abstract

pub_date

2019-02-14 00:00:00

pages

issue

issn

1546-0096

pii

10.1186/s12969-019-0309-6

journal_volume

pub_type

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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.