Contemporary diagnostic methods for hemophagocytic lymphohistiocytic disorders

Theodore S. Johnson, Joyce Villanueva, Alexandra H. Filipovich, Rebecca A. Marsh, Jack J. Bleesing

Research output: Contribution to journalReview article

29 Scopus citations

Abstract

Hemophagocytic lymphohistiocytosis is a life-threatening multi-system hyperinflammatory disorder characterized by dysfunctional cytolytic lymphocyte responses, hypercytokinemia, and widespread lymphohistiocytic tissue infiltration and destruction. Diagnosis and definitive therapy are often delayed as clinical efforts are directed toward treatment of presumed overwhelming infection. Sporadic cases occur in association with underlying immune dysfunction related to autoimmune disease, malignancy, or severe infection. However, familial cases predominate with remarkable associations between underlying genetic defects and dysregulation of immune responses. Here, we review the genetic and immunologic basis of contemporary diagnostic methods for hemophagocytic lymphohistiocytosis.

Original languageEnglish (US)
Pages (from-to)1-13
Number of pages13
JournalJournal of Immunological Methods
Volume364
Issue number1-2
DOIs
StatePublished - Feb 1 2011

Keywords

  • HLH
  • Hemophagocytic lymphohistiocytosis
  • Hemophagocytosis
  • Histiocytosis
  • Macrophage
  • T cell

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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