Skip to main content

Table 1 Diagnostic Criteria of HLH as defined by the HLH-2004 Criteria

From: Severe skin disease in lupus associated with hemophagocytic lymphohistiocytosis: case reports and review of the literature

HLH Criteria Case 1 Case 2
Fever ≥38.5 °C + +
Splenomegaly
Hemoglobin < 9 g/dLa +
Platelets < 100,000 microLa + +
Absolute Neutrophil Count < 1000/microLa
Hypertriglyceridemia: Fasting Triglycerides > 265 mg/dL + +
Hypofibrinogenemia: Fibrinogen < 150 mg/dL +
Hemophagocytosis in bone marrow, spleen, lymph node or liver +
Low or absent NK cell activity n/a n/a
Ferritin > 500 ng/mL + +
Elevated soluble CD25 (soluble IL-2 receptor alpha) two standard deviations above age-adjusted laboratory-specific norms +
  1. a Peripheral blood cytopenia as one criteria