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Diagnosis, Treatment, and Management of Hemophagocytic Lymphohistiocytosis in the Critical Care Unit

  • Melissa HinesEmail author
  • Neel Bhatt
  • Julie-An M. Talano
Chapter

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening condition characterized by uncontrolled inflammation. The management of HLH is challenging, in part, due to the multiple etiologies of the disease, the variations in presentations, and the rapidity of the progression of the disease. Due to the severity of their disease, these patients often require significant critical care support. HLH can be due to familial (genetic) causes or can be secondary to triggers such as infections, autoimmune disorders, and malignancy. Underlying conditions such as sepsis or malignancy could pose as major confounders while applying universal diagnostic criteria and therefore could lead to delay in diagnosis. This chapter focuses on the characteristics of the disease, pathophysiology, diagnostic criteria, treatment, and intensive care management of pediatric patients with HLH.

Keywords

Hemophagocytic lymphohistiocytosis (HLH) Macrophage activation syndrome (MAS) Intensive care unit (ICU) Multi-system organ failure Critical illness 

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Copyright information

© Springer International Publishing 2019

Authors and Affiliations

  • Melissa Hines
    • 1
    Email author
  • Neel Bhatt
    • 2
  • Julie-An M. Talano
    • 2
  1. 1.Department of Pediatric Medicine, Division of Critical CareSt. Jude Children’s Research HospitalMemphisUSA
  2. 2.Department of Pediatrics, Division of Pediatric Hematology/OncologyMedical College of Wisconsin, Children’s Hospital of WisconsinMilwaukeeUSA

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