
Figure 1. Hemophagocytosis in the bone marrow.
The diagnosis of HLH is based on clinical criteria. Diagnostic criteria that were developed by the Histiocyte Society for HLH (HLH-2004 criteria) are the most commonly used. They include:
Fever: ≥ 7 days of a temperature of at least 38.5°C (101.3°F)
Splenomegaly: a palpable spleen > 3 cm below the costal margin
Cytopenias: Counts must be below the specified range in at least two of the following cell lines:
Absolute neutrophils (< 1000/µL)
Platelets (< 100,000/µL)
Hemoglobin (< 9.0 g/dL)
Hypofibrinogenemia or hypertriglyceridemia
Fibrinogen level < 1.5 g/L or greater than three standard deviations below the age adjusted reference range value or
Fasting triglyceride level > 2 mmol/L or greater than three standard deviations above the age-adjusted reference range value
Natural killer cell activity is low or absent
Learn more about the diagnostic criteria for HLH.
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Cite this: Sarah K. Tasian. Fast Five Quiz: Hemophagocytic Lymphohistiocytosis Presentation and Diagnosis - Medscape - Jun 16, 2020.
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