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. Author manuscript; available in PMC: 2016 Apr 1.
Published in final edited form as: J Intensive Care Med. 2014 Jan 8;30(7):401–412. doi: 10.1177/0885066613517076

Table 3.

Diagnostic Criteria for HLH

A, Molecular diagnosis consistent with HLH
   OR
B, Fulfillment of 5/8 criteria:
1. Fever
      Temperature > 38.5 C for > 7 days
2. Splenomegaly
      Spleen tip palpated > 3cm below left costal margin
3. Cytopenias involving 2 or more lines
      Hb < 9 g/dL, or Plt < 100,000/uL, or ANC <1000/uL
4. Hypertriglyceridemia and/or hypofibrinogenemia
      fasting triglycerides > 2mmol/L, or >3SD above age-adjusted normal
      fibrinogen < 1.5 g/L, or < 3SD below age-adjusted normal
5. Hemophagocytosis in bone marrow, spleen or lymph nodes
6. Low or absent NK-cell activity
      determined by 51-Cr release assay
7. Ferritin > 500 ug/L (84% sensitivity)
      > 10,000 (90% sensitvity, 96% specificity)
8. soluble CD25 (IL-2 receptor) > 2400 U/mL
      > 2 SD above the age-adjusted mean

Adapted from: Henter JI, et al. Semin Oncol 1991;18:29-33

Henter JI, et al. Pediatr Blood Cancer 2007; 48:124-13