Sepsis-like multiorgan failure with fever, cytopenias, hepatitis/coagulopathy and hyperinflammation requiring structured HLH assessment, trigger search and early hematology-directed treatment.
Think HLH when an unexpectedly ill patient has persistent fever, falling blood counts, hepatitis/liver dysfunction, splenomegaly, coagulopathy or hypofibrinogenemia, hypertriglyceridemia and markedly elevated ferritin. No single ferritin threshold or bone-marrow finding is diagnostic.
Use HScore and/or HLH-2004 criteria as structured aids rather than absolute gatekeepers. Obtain ferritin trend, CBC, fibrinogen, triglycerides, liver tests, coagulation, LDH and soluble IL-2 receptor/other specialized tests when available; marrow evaluation may help identify malignancy or infection but hemophagocytosis is neither required nor specific.
Investigate infection (especially EBV and other viral/fungal causes), hematologic malignancy, rheumatologic/MAS phenotypes and drug/immune-therapy triggers. Do not stop antimicrobial/source-control evaluation just because HLH is suspected.
Caution
Do not diagnose or exclude adult HLH from ferritin alone, and do not wait for marrow hemophagocytosis before treating a rapidly deteriorating high-probability syndrome.
Current adult suspected-HLH diagnostic and investigation framework.
Adult HLH management recommendations including trigger-directed and immunomodulatory therapy.
Evidence status
Review metadata not yet recorded.
Status, review dates and guideline versions appear here once editorial metadata is authored for this content.