Haemophagocytic syndrome during pregnancy: a case report and review of literature

Figures & data

Table 1. Scoring systems for the diagnosis of HLH.

Symptom	HLH-2004	HScore
Fever	0 (<38.5) or 1 (≥38.5)	0 (<38.4), 33 (38.4–39.4), or 49 (>39.4)
Hepatosplenomegaly	0 (No) or 1 (Yes)	0 (none), 23 (hepatomegaly), or 38 (hepatomegaly)
Cytopenia	0 (one series) or 1 (two or three departments)	0 (one department), 24 (two departments), or 34 (three departments)
Ferritin	0 (<500) or 1 (≥500)	0 (<2000), 35 (2000–6000), or 50 (>6000)
Triglycerides	0 (<3) or 1 (≥3)	0 (<1.5), 44 (1.5–4), or 64 (>4)
Fibrinogen	0 (>1.5) or 1 (≤1.5)	0 (>2.5) or 30 (≤2.5)
Bone marrow blood phagocytosis	0 (No) or 1 (Yes)	0 (No) or 35 (Yes)
Aspartate aminotransferase	–	0 (<30) or 19 (≥30)
Known potential immunosuppression	–	0 (No) or 18 (Yes)
Scores^a	≥4 (100%)^b	90 (>1%)^c 100 (1%) 110 (3%) 120 (5%)
		130 (9%) 140 (16%) 150 (25%) 160 (40%)
		170 (54%) 180 (70%) 190 (80%) 200 (88%)
		210 (93%) 220 (96%) 230(98%) 240 (99%)
		250 (>99%)

HLH: haemophagocytic lymphohistiocytosis.

^aThe sum of the above nine symptoms scores.

^b100% indicates a definite diagnosis.

^cThe probability of disease occurrence corresponding to the score.