Table 2.
Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis | |||
---|---|---|---|
Criterion | Patient 1 | Patient 2 | Patient 3 |
Fever (Temperature>38.5C for >7 days) | Yes | Yes | Yes |
Splenomegaly | no | No | Yes |
Cytopenias involving two or more lines | yes | Yes | Yes |
Hypertriglyceridemia and/or hypofibrinogenemia (fasting TG's levels>2mmol/liter; fibrinogen<1.5g/liter) | Yes | Yes | Yes |
(hypofibrinogenemia) | (both) | (hypofibrinogenemia) | |
Ferritin level>500mcg/L | Yes | Yes | Yes |
Soluble CD25 level>2400 U/mL (ie, sIL2R) | Not measured | Yes | Not measured |
Decreased or absent natural killer cell activity | Not measured | Yes | Not measured |
Hemophagocytosis in bone marrow, central nervous system, or lymph nodes | Yes | Yes | Yes |