Table 1.

Clinical and laboratory parameters of the HScore with the corresponding points

HScore for secondary HLH
Clinical/laboratory parameters	Points
Temperature (°C)
< 38.4	0
38.4–39.4	33
> 39.4	49
Organomegaly
None	0
Hepatomegaly or splenomegaly	23
Hepatomegaly and splenomegaly	38
Number of cytopeniasa
One lineage	0
Two lineages	24
Three lineages	34
Triglycerides (mmol/L)
< 1.5	0
1.5–4.0	44
> 4.0	64
Fibrinogen (g/L)
> 2.5	0
≤ 2.5	30
Ferritin (ng/mL)
< 2000	0
2000–6000	35
> 6000	50
Serum aspartate aminotransferase (U/L)
< 30	0
≥ 30	19
Hemophagocytosis on bone marrow aspirate
No	0
Yes	35
Known immunosuppressionb
No	0
Yes	18

HLH hemophagocytic lymphohistiocytosis

^aDefined as either haemoglobin concentration of 9.2 g/dL or less, a white blood cell (WBC) count of 5000/mm³ or less, or platelet count of 110.000/mm³ or less

^bHIV positive or receiving long-term immunosuppressive therapy (glucocorticoids, cyclosporine, azathioprine etc.)