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Table 1 Clinical features of study patients at the time of initial diagnosis and time of identification of refractoriness

From: PEG-aspargase and DEP regimen combination therapy for refractory Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis

Clinical features

Initial diagnosis

refractoriness

Number (n = 28)/percentage (%)

Number (n = 28)/percentage (%)

Fever

28/100.0

28/100.0

Neutrophils <1 × 109/L

15/53.6

13/46.4

Hgb <90 g/L

16/57.1

18/64.3

PLT <100 × 109/L

23/82.1

25/89.3

TG >3 mmol/L

17/60.7

18/64.3

Fgb <1.5 g/L

10/35.7

16/57.1

Ferritin ≥500 μg/L

21/75.0

25/89.3

ALT >40 U/L

24/85.7

24/85.7

TBiL >17.1 μmol/L

16/57.1

18/64.3

LDH >190 U/L

18/64.3

21/75.0

Splenomegaly

28/100.0

28/100.0

Hemophagocytosis

21/75.0

22/78.6

Decline of NK cell activity

17/60.7

18/64.3

Elevated soluble CD25

25/89.3

28/100.0

Positive EBV-DNA in whole blood

28/100.0

28/100.0

Positive EBER in lymph node/bone marrow

3/10.7

4/14.3

  1. Hgb hemoglobin, PLT platelet, Fgb fibrinogen, ALT alanine aminotransferase, TBiL total bilirubin, LDH lactic dehydrogenase