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Hemophagocytic Lymphohistiocytosis

Teaching Points

  • Definition: severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages
  • Presentation: fever, hepatosplenomegaly, lymphadenopathy, jaundice and a rash.
  • Diagnosis: Fulfillment of five out of the eight criteria below: fever (>100.4 °F, >38 °C), splenomegaly, cytopenias affecting at least two of three lineages, hypertriglyceridemia, ferritin ≥ 500 ng/ml, haemophagocytosis in the bone marrow, spleen or lymph nodes, low or absent natural killer cell activity, soluble CD25
  • Treatment:  high dose corticosteroids, etoposide and cyclosporin

PowerPoint: Daily Report 9-16-2016

Further Reading
Approach to Hemophagocytic Syndromes

 

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