Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO)

Michael B. Jordan, Carl E. Allen, Jay Greenberg, Michael Henry, Michelle L. Hermiston, Ashish Kumar, Melissa Hines, Olive Eckstein, Stephan Ladisch, Kim E. Nichols, Carlos Rodriguez-Galindo, Birte Wistinghausen, Kenneth L. McClain

Research output: Contribution to journalReview articlepeer-review

113 Scopus citations

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, often associated with genetic defects of lymphocyte cytotoxicity. Though a distinctive constellation of features has been described for HLH, diagnosis remains challenging as patients have diverse presentations associated with a variety of triggers. We propose two concepts to clarify how HLH is diagnosed and treated: within the broader syndrome of HLH, “HLH disease” should be distinguished from “HLH disease mimics” and HLH subtypes should be categorized by specific etiologic associations, not the ambiguous dichotomy of “primary” and “secondary.” We provide expert-based advice regarding the diagnosis and initiation of treatment for patients with HLH, rooted in improved understanding of its pathophysiology.

Original languageEnglish
Article numbere27929
JournalPediatric Blood and Cancer
Volume66
Issue number11
DOIs
StatePublished - 1 Nov 2019
Externally publishedYes

Keywords

  • hematology
  • hemophagocytic lymphohistiocytosis
  • immunology

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