Table 2.

Pertinent Diagnostic Criteria, Categorized by Patient’s Results

Features	HLH-2004 Criteria for HLH	Criteria for Adult-Onset Still’s Disease		Criteria for Overt DIC		Society for Maternal-Fetal Medicine Criteria for Amniotic Fluid Embolism
		Yamaguchi	Fautrel	ISTH Score	Modified ISTH for Pregnancy (3)
Requirement for diagnosis	At least five criteria	At least five criteria, with at least 2 major	Either four major, or three major and two minor	At least 5 points	At least 3 points	All four criteria
Diagnosis satisfied?	Yes: 5	Yes: 5, 3 major	Yes: 4 major, 1 minor	No: 4	No: 2	No: 0
Criteria satisfied	Fever ≥38.5°CSplenomegalyBicytopenia (at least 2 of: hemoglobin <9 g/dL, Plt <100,000/μL, neutrophils <1,000/μL) Triglycerides >265 mg/dL and/or fibrinogen <150 mg/dL Ferritin >500 ng/mL	Fever ≥39°C at least 1 wk Arthralgias at least 2 wk Typical rash during feverAbnormal liver function tests Hepatomegaly and/or splenomegaly	Fever ≥ 39 C Arthralgias Transient erythema Neutrophils ≥ 80% Maculopapular rash	Plt < 100,000/μL (1 point), <50,000/μL (2 points) d-dimer “moderate increase” (2 points) or “severe increase” (3 point)	Plt < 100,000/μL (1 point), <50,000/μL (2 points) Fibrinogen < 200 mg/dL (1 point)
Criteria not satisfied	[Hemophagocytosis in biopsy of bone marrow, spleen, lymph node, or liver] [Low natural killer cell activity] [Elevated soluble interleukin-2 receptor]	Leukocytes ≥ 10,000/μL with ≥ 80% neutrophils Pharyngitis Lymphadenopathy Both antinuclear antibody and rheumatoid factor negative	Leukocytes ≥ 10,000/μL Pharyngitis[Glycosylated ferritin < 20%]	PT prolonged by 3-6 s (1 point), ≥ 6 (2 points)Fibrinogen < 100 mg/dL (1 point)	PT prolonged by 25-50% above normal (1 point), >50% (2 points)	Sudden, not gradual, onset of hypotension and respiratory failure Onset during labor or within 30 min of delivery Overt DIC No fever during labor

DIC = disseminated intravascular coagulation, HLH = hemophagocytic lymphohistiocytosis, ISTH = International Society on Thrombosis and Haemostasis, Plt = platelets, PT = prothrombin time

Italics indicate minor criteria; normal text indicates major criteria. Square brackets indicate tests not performed.