Clinical characteristics and prognostic factors of 75 cases with acquired hemophagocytic syndrome

Figures & data

Table 1. HLH-2004 diagnostic indexs occurrence rate.

Diagnostic index	Distribution	Cases	Occurrence number(%)
SF ≥ 500ug/L	[1500(1487.18, 1500)]	65	62(95.38%)
persistent fever above 38.5°C	61/75	75	61(81.33%)
sCD25 ≥ 2400U/ml	[7500(2758, 8602.48)]	28	21(75.00%)
Hemophagocytic cells were seen in bone marrow aspirates	38/56	56	38(67.86%)
Enlargement of the spleen(or/and liver)	47/75	75	47(62.66%)
Cytopenia of at least two lineages	40/75	75	40(53.33%)
NK cell activity was decreased or absent (<7%)	[7.47(2.87,18.59)]	30	14(46.67%)
FIB < 1.5 g/L	[1.61(0.99,2.67)]	73	34(46.57%)
TG ≥ 3 mmol/L	[2.73(1.99,3.51)]	72	29(40.27%)

Table 2. Comparison of clinical characteristics and laboratory tests.

Project	High SII group (n = 36)	Low SII group (n = 38)	χ^2/Z/t value	P value
Red cell count(×10^12/L)	3.24 ± 0.69	2.87 ± 0.89	1.993^c	0.050
White cell count(×10^9/L)	4.60(2.54,8.02)	2.76(1.01,4.18)	2.790^b	0.005
Platelet Count(×10^9/L)	80.50(53,131.25)	29(13.75,68.75)	4.062^b	0.001
Neutrophil count(×10^9/L)	3.49(1.66,6.74)	1.18(0.31,2.41)	4.207^b	0.001
Total bilirubin(umol/L)	12.20(8.25,21.38)	24.45(12.10,49.38)	2.250^b	0.024
Direct bilirubin(umol/L)	5.25(3.83,11.73)	14.55(6.30,29.80)	2.206^b	0.027
LDL (mmol/L)	1.93(1.42,2.30)	1.51 ± 0.75	2.281^b	0.023
Fibrinogen(g/L)	1.77(1.32,3.72)	1.38(0.82,2.34)	2.335^b	0.020
CD4 + cell activity (%)	33.54 ± 14.71	20.02 ± 10.25	2.775^c	0.010
survival time (month)	3.43(0.87,14.70)	0.84(0.35,5.28)	1.959^b	0.050

Note: ^a shows chi-square test; ^b shows non-parametric rank-sum test; ^c shows t test; ^d shows Fisher's exact probability test; ^e is the corrected chi-square test; ^f represents the corrected t test.

Table 3. Outcomes of the initial hospitalization.

Classification of Etiology	Follow the HLH-2004 protocol (n)	Cases	The rate of improvement(%)
Tumor-associated HPS	14	25	8(32.00%)
Infection-related HPS	20	34	11(32.35%)
Autoimmune HPS	9	14	5(35.71%)
HPS of unknown etiology	0	2	1(50.00%)

Figure 1. Survival Curve of High SII Group and Low SII Group.

Table 4. Comparison of clinical characteristics.

Project	Poor prognosis group(n = 32)	Better prognosis group(n = 40)	χ^2/Z/t value	P value
Male/female (n/n)	13/19	14/26	0.240^a	0.624
Age of onset (years)	66(56.25,72)	56.5(32,65)	2.756^b	0.006
Fever >38.5 ° C (n)	29(90.60%)	30(75.00%)	2.934^a	0.087
Hepatosplenomegaly/splenomegaly/hepatomegaly/none (n)	1/16/1/14	4/23/0/13	3.036^d	0.372
Enlarged lymph nodes (n)	21(65.60%)	20(50.00%)	1.770^a	0.183
Rash (n)	4(12.50%)	7(17.50%)	0.066^e	0.798

Note: ^a shows chi-square test; ^b shows non-parametric rank-sum test; ^c shows t test; ^d shows Fisher's exact probability test; ^e is the corrected chi-square test; ^f represents the corrected t test.

Table 5. Comparison of laboratory tests.

Project	Poor prognosis group(n = 32)	Better prognosis group(n = 40)	χ^2/Z/t value	value
Red cell count(×10^12/L)	2.69 ± 0.93	3.29 ± 0.60	3.293^c	0.002
White cell count(×10^9/L)	2.94(1.10,6.13)	3.81(2.50,5.97)	1.388^b	0.165
Platelet Count(×10^9/L)	29(14.75,72)	71.50(48.50,131)	3.401^b	0.001
Hemoglobin(g/L)	81.56 ± 23.67	95.56 ± 19.18	2.772^c	0.007
Neutrophil count(×10^9/L)	1.70(0.44,5.02)	2.35(1.21,4.49)	0.844^b	0.399
SII	215.38(33.52,770.27)	282.54(29.14,685.24)	0.174^b	0.862
CRP(mg/L)	18.20(8.73,50.11)	13.30(4.40,62.93)	0.801^b	0.423
ALT(U/L)	56(34,126)	75(36.90,254.65)	0.841^b	0.401
AST(U/L)	75(43.60,187)	81.50(29.90,299.73)	0.226^b	0.821
LDH(U/L)	663(381,1127)	482(310,1087.75)	0.765^b	0.444
Total bilirubin(umol/L)	33.40(17.40,57.70)	12.20(8.70,19.65)	3.339^b	0.001
Direct bilirubin(umol/L)	21.20(7,50.60)	5.45(3.50,9.48)	3.264^b	0.001
Indirect bilirubin(umol/L)	10.70(6.60,16.10)	6.65(4.50,9.48)	2.615^b	0.009
Albumin(g/L)	25.60 ± 4.48	29.28 ± 4.28	3.555^c	0.001
Globulin(g/L)	23.50(22,26.50)	28.34 ± 6.70	3.131^b	0.002
Triglycerides(mmol/L)	2.85(2.03,3.48)	2.35(1.97,3.43)	0.517^b	0.605
HDL(mmol/L)	0.51(0.24,0.77)	0.55(0.35,0.99)	1.198^b	0.231
LDL(mmol/L)	1.58(0.88,2.05)	1.70(1.37,2.05)	0.930^b	0.352
β2 microglobulin(mg/L)	6.39 ± 2.49	4.94(3.46,6.26)	1.397^b	0.163
SF(ug/l)	1500(1500,1500)	1500(1141.40,1500)	1.093^b	0.274
Fibrinogen(g/L)	1.36(0.82,2.40)	1.73(1.23,2.80)	1.898^b	0.058
FDP(ug/ml)	11(5.70,22.70)	13.35(5.20,22.98)	0.072^b	0.942
D-dimer(mg/L)	4.21(2.36,9.37)	4.63(1.60,8.71)	0.823^b	0.410
sCD25(U/ml)	7500(7151.50,8402.95)	6331.50(1578,10905)	0.986^b	0.324
CD4 + cell(%)	24.53 ± 11.92	26.51 ± 16.17	0.336^c	0.739
CD8 + cell(%)	37.39 ± 17.97	28.53 ± 21.88	1.072^c	0.294
NK cell(%)	19.02 ± 15.87	6.97(2.54,17.14)	1.486^b	0.137
Hemophagocytosis in bone marrow(n)	13(68.40%)	23(65.70%)	0.041^a	0.840
EBV nucleic acid positive(n)	7(77.80%)	4(23.50%)	– – ^d	0.014
Follow the HLH-2004 (n)	13(40.60%)	27(67.50%)	5.200^a	0.023

Note: ^a shows chi-square test; ^b shows non-parametric rank-sum test; ^c shows t test; ^d shows Fisher's exact probability test; ^e is the corrected chi-square test; ^f represents the corrected t test.

Figure 2. ROC Curves for Prognostic Factors.