From: Recent advances in the diagnosis and treatment of hemophagocytic lymphohistiocytosis
A | B (five out of eight criteria have to be fulfilled) |
---|---|
Molecular diagnosis consistent with HLH | Fever |
 | Splenomegaly |
 | Cytopenias in two out of three cell lines (hemoglobin <9 mg/dl (<10 mg/dl in infants <4 weeks old), platelets <100,000/μl, neutrophils <1,000/μl) |
 | Hypertriglyceridemia (fasting triglycerides ≥265 mg/dl) and/or hypofi brinogenemia (fibrinogen ≤1.5 g/l) |
 | Hemophagocytosis in bone marrow, spleen, lymph nodes, or liquor |
 | Ferritin >500 ng/ml |
 | sCD25 >2,400 U/ml |
 | Decreased or absent NK cell cytotoxicity |