Table 2

Diagnostic criteria 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


Either A or B have to be fulfilled for a diagnosis of hemophagocytic lymphohistiocytosis (HLH). Additional laboratory and clinical findings may include lymphadenopathy, symptoms of central nervous system involvement, exanthema, jaundice, edema, transaminase abnormalities, hypoproteinemia, hyponatremia, increased very low density lipoproteins, decreased high density lipoproteins, liver biopsy consistent with chronic persistent hepatitis. sCD25, soluble CD25; NK, natural killer. Adapted from [1,89,90].

Bode et al. Arthritis Research & Therapy 2012 14:213   doi:10.1186/ar3843