Hemophagocytic lymphohistiocytosis

Flavia G.N. Rosado, Annette S. Kim

Research output: Contribution to journalReview articlepeer-review

183 Scopus citations

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a frequently fatal and likely underdiagnosed disease involving a final common pathway of hypercytokinemia, which can result in end-organ damage and death. Although an early diagnosis is crucial to decrease mortality, the definitive diagnosis is often challenging because of the lack of specificity of currently accepted diagnostic criteria and the absence of confirmatory gold standards. Because of the wide range of laboratory assays involved in the diagnosis of HLH, practicing pathologists from a broad spectrum of clinical specialties need to be aware of the disease so that they may appropriately flag results and convey them to their clinical counterparts. Our article summarizes these new advances in the diagnosis of HLH and includes a review of clinical findings, updated understanding of the pathogenesis, and promising new testing methods.

Original languageEnglish (US)
Pages (from-to)713-727
Number of pages15
JournalAmerican journal of clinical pathology
Volume139
Issue number6
DOIs
StatePublished - Jun 2013
Externally publishedYes

Keywords

  • Acquired
  • Genetic
  • Hemophagocytic lymphohistiocytosis
  • Hypercytokinemia
  • Macrophage activation syndrome
  • Perforin

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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