Hemophagocytic lymphohistiocytosis (HLH): A review of literature

Rohtesh S. Mehta, Roy E. Smith

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64 Scopus citations


Hemophagocytic lymphohistiocytosis (HLH) is a rare disease in children and an exceptionally rare occurrence in adults. It is categorized broadly into primary (familial) or the secondary types; the latter being associated most commonly with an underlying malignancy. HLH carries a high rate of mortality, and the treatment itself is associated with significant morbidity and risk of mortality. A high degree of suspicion for the diagnosis, early treatment and aggressive supportive care is critical for management. We present a comprehensive review of literature describing the clinical features, diagnosis, management and outcome of HLH.

Original languageEnglish (US)
Article number740
JournalMedical Oncology
Issue number4
StatePublished - Dec 2013
Externally publishedYes


  • Cytopenia
  • Fever of unknown origin (FUO)
  • Hemophagocytic lymphohistiocytosis (HLH)
  • Hyperferritinemia
  • Hypofibrinogenemia
  • Soluble CD25


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