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

Pediatric Rheumatology Online Journal

Leslie A. Benson¹  Mark Gorman¹  Jenny Chan Yuen²  Alison Mann²  Amy Starmer²  Patricia Meleedy-Rey²  Taruna Banerjee²  Gregory P. Priebe³  Jared Rowe⁴  Barbara A. Degar⁴  Leslie Lehmann⁴  Michelle L. Schoettler⁴  Marc A. Schwartz⁴  Katja G. Weinacht⁴  Craig D. Platt⁵  Ezgi H. Baris⁵  Robert P. Sundel⁵  Fatma Dedeoglu⁵  Peter A. Nigrovic⁵  Melissa M. Hazen⁵  Olha Halyabar⁵  Margaret H. Chang⁵  Mindy S. Lo⁵  Catherine M. Biggs⁵  Lauren A. Henderson⁵  Neeraj K. Surana⁶ 

[1] Department of Neurology, Boston Children’s Hospital;Department of Pediatrics, Boston Children’s Hospital;Division of Critical Care Medicine, Boston Children’s Hospital;Division of Hematology-Oncology, Boston Children’s Hospital;Division of Immunolgy, Boston Children’s Hospital;Division of Infectious Diseases, Boston Children’s Hospital;

关键词: Macrophage activation syndrome (MAS);    Hemophagocytic lymphohistiocytosis (HLH);    Quality improvement research;    Evidence-based guideline;   

DOI  :  10.1186/s12969-019-0309-6

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown