Table 1.
Diagnostic and classification criteria for sHLH/MAS.
| HLH | MAS | ||||
|---|---|---|---|---|---|
| 2004 HLH8 | 2014 HScore9 | 2009 MAS/jSLE10 | 2016 EULAR MAS/sJIA11 | 2019 MAS/sJIA (MS) score12 | |
| Fever (°C) | ≥38.5 | 0 (<38.4), 33 (38.4–39.4), or 49 (>39.4) | ≥38 | Not specified | – |
| Hyperferritinemia (ng/mL) | ≥500 | 0 (<2000), 35 (2000–6000), or 50 (>6000) | >500 | >684 | 0.0001×value |
| Hypertriglyceridemia (mg/dL) | ≥265 | 0 (<132.7), 44 (132.7-354), or 64 (>354) | >178 | >156 | – |
| Hypofibrinogenemia (g/L) | ≤1.5 | 0 (>2.5) or 30 (≤2.5) | ≤1.5 | ≤3.6 | -0.004×value |
| Cytopenia | Platelets<100×109/L, Hemoglobin<90 g/L, Neutrophils<1.0×109/L | Platelets<110×109/L, Hemoglobin<92 g/L, Leukocytes<5.0×109/L | Platelets<150×109/L, Hemoglobin<90 g/L, Leukocytes<4.0×109/L | Platelets≤181×109/L, | -0.003×platelets |
| Aspartate aminotransferase (AST) (U/L) | – | 0 (<30) or 19 (≥30) | >40 | >48 | – |
| Lactate dehydrogenase (LDH) (U/L) | – | – | >567 | – | 0.001×value |
| NK cell activity | Low or absent | – | – | – | |
| Soluble IL-2Rα (sCD25) (U/mL) | ≥2400 | – | – | – | |
| Organomegaly | Splenomegaly | 0 (N), 23 (hepato- or splenomegaly), or 38 (hepato- and splenomegaly) | Hepato- and splenomegaly (≥3 cm below the costal arch) | – | – |
| Hemorrhagic manifestations | – | – | Purpura, easy bruising, or mucosal bleeding | – | 1.54×1(Y) or ×0(N) |
| Central nervous system involvement | – | – | Irritability, disorientation, lethargy, headache, seizures, or coma | – | 2.44×1(Y) or ×0(N) |
| Active arthritis | – | – | – | -1.3×1(Y) or ×0(N) | |
| Known immunosuppression | – | 0 (N) or 18 (Y) | – | – | |
| Hemophagocytosis | Bone marrow, spleen, or lymph nodes | 0 (N) or 35 (Y) in bone marrow | Bone marrow aspirate | – | – |
| Diagnosis | Presence of genetic mutations or 5 of 8 criteria met | Sum of parameters ≥169 | At least 1 clinical criterion + 2 laboratory criteria | Sum of parameters ≥-2.1 | Fever in known or suspected sJIA + ferritin + 2 of 4 remaining criteria met |