How do you work-up the patient with suspected HLH?

To Determine Diagnosis
  • Clinical history and findings
  • Use of Clinical Scores:
    • HLH Diagnostic Criteria (primary HLH)
    • H-Score (secondary HLH)
  • Laboratory evaluation:
    • FBC
    • Ferritin
    • Fasting triglycerides
    • Coagulation screen (fibrinogen, PT, aPTT)
    • LFTs, LDH, albumin
    • Immunological testing
    • Bone marrow biopsy
Definitive testing:
If suspected, do not wait to treat!
  • ADAMTS13 activity
  • Anti-ADAMTS13 Ab
  • Sequencing of ADAMTS13 gene
To Determine Aetiology
  • Infection work-up:
    • Relevant imaging
    • Bacterial and viral studies
  • Cancer work-up:
    • PET-CT
  • Tissue biopsy
  • Tumour markers
Autoimmune panel
  • Genetic testing