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

Halyabar O., Chang M. H., Schoettler M. L., Schwartz M. A., Baris E. H., Benson L. A., ...More

Pediatric rheumatology online journal, vol.17, no.1, pp.7, 2019 (SCI-Expanded, Scopus) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 17 Issue: 1
  • Publication Date: 2019
  • Doi Number: 10.1186/s12969-019-0309-6
  • Journal Name: Pediatric rheumatology online journal
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.7
  • Keywords: Macrophage activation syndrome (MAS), Hemophagocytic lymphohistiocytosis (HLH), Quality improvement research, Evidence-based guideline, JUVENILE IDIOPATHIC ARTHRITIS, ONSET STILLS-DISEASE, INTERFERON-GAMMA, RHEUMATOID-ARTHRITIS, CASE SERIES, T-CELLS, INTERLEUKIN-18, SECONDARY, ANAKINRA, FERRITIN
  • Marmara University Affiliated: No

Abstract

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.