HLH associated with malignancy


  • similar to CRS
  • malignancy, infection in malignancy, treatment can all cause HLH
  • in cancer patients with active therapy
  • HLH in autoimmune flavor (scleroderma) => call it macrophage activation syndrome
  • very high ferritin (>15,000)
  • elevated IL2 receptor with positive CD25 subunit
  • low fibrinogen
  • important to exclude active infection (2ndary HLH) prior targeted therapy (very high risk)
    • etoposide +/- steroids, IL6, complement suppression etc.1

Footnotes

  1. CHEST fellow course 2024 Heme Onc Emergencies