Table 1.
Diagnostic guidelines for hemophagocytic syndrome (HPS) or hemophagocytic lymphohistiocytosis (HLH)
| Clinical/laboratory findings | HLH-2004 | HScorec | MH scorec,d | sJIA/MAS |
|---|---|---|---|---|
| Fever (°C) | ≥ 38.5 |
< 38.4: 0 points 38.4–39.4: 33 points > 39.4: 49 points |
− | + |
| Organomegaly | Splenomegaly |
Absent: 0 points Hepatomegaly or splenomegaly: 23 points Hepatomegaly and splenomegaly: 38 points |
Splenomegaly: Absent: 0 points Present: 12 points |
− |
| Cytopenia |
Cytopenia of ≥ 2 series Hemoglobin < 9 g/dL Platelet count < 100 × 109/L Absolute neutrophil count < 1×109/L |
Single series: 0 points Two series: 24 points Three series: 34 points |
Neutrophil count > 1.4: 0 points ≤ 1.4, ×109/L: 37 points Platelet: > 78: 0 points ≤ 78, ×109/L: 11 points Hemoglobin > 8.3: 0 points ≤ 8.3, g/dL: 11 points |
Platelet count ≤ 181 × 109/L |
| Triglyceridesa (mmol/L) |
Hypertriglycemia and/or hypofibrinogenemia Triglycerides ≥ 3,0 (65 mg/dL) |
< 1.5: 0 points 1.5–4. 0: 44 points > 4.0: 64 points |
> 156 mg/dl | |
| Fibrinogen (mg/dL)a | Fibrinogen ≤ 150 mg/dl, (1.5 g/l) |
> 2.5: 0 points ≤ 2.5: 30 points |
> 131: 0 points ≤ 131 mg/dl: 15 points (1.31 g/l) |
≤ 360 mg/dl |
| Ferritin (ng/L) | ≥ 500 μg/L |
< 2000: 0 points 2000–6000: 35 points > 6000: 50 points |
> 684 ng/ml | |
| Serum AST (IU/L) | − |
<30: 0 points ≥ 30: 19 points |
> 48 U/L | |
| Hemophagocytosis in the bone marrow | Hemophagocytosis in the bone marrow, spleen or the lymph nodes |
Absent: 0 points Present: 35 points |
||
| Immunosupressionb | − |
Absent: 0 points Present: 18 points |
||
| NK cell activitiy | Low or absent | − | ||
| sCD25 (sIL2Rα) | ≥ 2400 U/ml | − | ||
| Age at onset (years) | − | − |
> 1.6: 0 points ≤ 1.6: 37 points |
− |
aAre used as single parameters for HLH-2004 scoring
bThe presence of human immune deficiency virus (HIV) infection or long term treatment with immunosuppressive drugs such as glucocorticoids, cyclosporine or azathioprine
cA scoring is applied. An HScore of ≥ 169 has 93% sensitivity and 86% specificity for HLH. A cut-off value of ≥ 60 for MH score discriminated best between pHLH and MAS
dIn a sJIA patient with fever and Ferritin > 684 ng/ml, the presence of two additional parameters has a sensitivity of 73% and specificity of 99% for this score