Critical care education one page at a time. Simple, free, & open source.
Nick Mark’s OnePager guides to critical care topics.
Hemophagocytic lymphohistiocytosis (HLH)
#pulmonary #rheumatology #criticalCare #hematology
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome due to aberrant activation of macrophages. It can be challenging to diagnose & often leads to critical illness with multiple organ system failure. This ICU OnePager reviews the etiology, diagnostic criteria, and treatment approach to this complex illness.
Current version 1.1 (originally posted 2022-12-15).
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Al Nasrallah et al, Hemophagocytic Lymphohistiocytosis in the Medical ICU: A Single-Institution Cohort Study on Acute Liver Failure and Mortality, Critical Care Explorations 2021
Rosée et al, Recommendations for the management of hemophagocytic lymphohistiocytosis in adults, Blood 2019 (this is the definitive set of treatment guidelines for HLH; highly recommended reading)
Miao et al, Pathogenic Gene Mutations or Variants Identified by Targeted Gene Sequencing in Adults With Hemophagocytic Lymphohistiocytosis, Frontiers of Immunology 2019
Hayden et al, Soluble interleukin-2 receptor is a sensitive diagnostic test in adult HLH, Blood Advances 2017
Lee et al, Natural Killer Cell Function Tests by Flowcytometry-Based Cytotoxicity and IFN-γ Production for the Diagnosis of Adult Hemophagocytic Lymphohistiocytosis, International Journal of Molecular Science 2019
Schram et al, Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7 years, British Journal of Hematology 2016
Grom et al, Macrophage activation syndrome in the era of biologic therapy, Nature Reviews Rheumatology 2016
Schram et al, Marked hyperferritinemia does not predict for HLH in the adult population, Blood 2015
Zerah et al, Cutaneous findings in hemophagocytic lymphohistiocytosis, Dermatology 2015
Karras et al, What nephrologists need to know about hemophagocytic syndrome, Nature Reviews Nephrology 2009
Henter et al, HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis, Pediatric Blood Cancer 2007
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None… Yet!
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Current version 1.0 (originally posted 2022-12-14).
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RheumOnePagers - an excellent site for all things rheumatology!