Hemophagocytic Lymphohistiocytosis: Lessons Learned from the Dark Side

Research output: Contribution to journalReview articlepeer-review

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe form of immune dysregulation often presenting as unremitting fever, cytopenia, hepatosplenomegaly, coagulopathy, and elevation of typical HLH biomarkers. HLH is universally fatal, if left untreated. The HLH-2004 criteria are widely used to diagnose this condition, but there is growing concerns across different settings that its application may result in undertreatment of certain patients. There is an expanding spectrum of genetic conditions that can be complicated by HLH. This review summarizes the current concepts in HLH, the lessons learned from the past, and provide an overview of the latest diagnostic and treatment modalities.

Original languageEnglish (US)
Pages (from-to)485-497
Number of pages13
JournalImmunology and Allergy Clinics of North America
Volume40
Issue number3
DOIs
StatePublished - Aug 2020

Keywords

  • Flow cytometry-based primary immunodeficiency disorder screening tests
  • Hematopoietic cell transplantation
  • Hemophagocytic syndrome
  • Hyperinflammation
  • Primary immunodeficiency
  • Secondary hemophagocytic lymphohistiocytosis
  • Targeted therapy

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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