噬血细胞性淋巴组织细胞增生症与脓毒症临床特征比较

doi:10.12372/jcp.2024.22e1731

Abstract

Abstract:

Objective To compare the clinical features of hemophagocytic lymphohistiocytosis (HLH) and sepsis, and to find laboratory markers to help differential diagnosis. Methods The clinical data of HLH children without underlying disease or sepsis children with fever and decreased peripheral blood two cell lines or splenomegaly from January 2013 to December 2020 was retrospectively analyzed. Results A total of 106 patients were enrolled in this study. There were 72 children (46 boys and 26 girls) in the HLH group, and the median age was 26.5 (14.3-56.8) months. There were 34 children (18 boys and 16 girls) in the sepsis group, and the median age was 14.0 (3.8-89.0) months. The total length of hospital stay and PICU stay in the sepsis group were longer than those in the HLH group, and the differences were statistically significant (P<0.05). Compared with the sepsis group, the white blood cell count (WBC)-Min, absolute neutrophil count (ANC)-Min, hemoglobin (HB)-Min, platelet count (PLT)-Min, creatinine (CREA)-Max, fibrinogen (FIB)-Min, C-reactive protein (CRP)-Max, and procalcitonin (PCT)-Max were lower, and triglyceride-Max and serum ferritin (SF)-Max were higher in the HLH group. The duration of HB reduction was shorter in the HLH group, while the duration of ANC reduction was longer, and the differences between the two groups were statistically significant (all P<0.05). Multivariate logistic regression analysis showed that the decreased levels of WBC-Min, FIB-Min, CREA-Max and PCT-Max were helpful for the diagnosis of HLH (P<0.05). The AUC of SF-Max to aid in the diagnosis of HLH was 0.72 (95%CI: 0.62-0.80), according to ROC curve analysis. The combined predictors of WBC-Min, FIB-Min, CREA-Max and PCT-Max were obtained by logistic regression equation fitting, and the AUC of which in the diagnosis of HLH was 0.95 (95%CI: 0.89-0.99). The AUC of the combined predictors was significantly larger than that of SF-Max, and the difference was statistically significant (P<0.01). Conclusions The fitting of WBC-Min, FIB-Min, CREA-Max and PCT-Max may help to better distinguish HLH from sepsis.

Key words: hemophagocytic lymphohistiocytosis, sepsis, serum ferritin, clinical characteristics

ZHONG Xiaomei, REN Hong, ZHANG Jian, LI Biru, QIAN Juan, NING Botao, GAO Yijin, WANG Ying. Comparison of clinical features between hemophagocytic lymphohistiocytosis and sepsis[J].Journal of Clinical Pediatrics, 2024, 42(6): 547-552.

Figures/Tables 6

References 19

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项目	HLH组(n=72)	脓毒症组(n=34)	统计量	P
男性[n(%)]	46(63.9)	18(52.9)	χ²=1.16	0.282
年龄[M(P₂₅~P₇₅)]/月	26.5(14.3~56.8)	14.0(3.8~89.0)	Z=1.47	0.143
总住院时间[M(P₂₅~P₇₅)]/d	15.5(9.5~26.8)	31.0(14.3~39.5)	Z=2.65	0.008
PICU入住时间[M(P₂₅~P₇₅)]/d	3.5(0~10.0)	14.5(5.0~25.5)	Z=4.73	<0.001
治疗结局[n(%)]
存活	48(66.7)	22(64.7)	χ²=0.04	0.842
死亡	24(33.3)	12(35.3)

实验室指标	HLH组(n=72)	脓毒症组(n=34)	统计量	P
WBC-Min[M(P₂₅~P₇₅)]/×10⁹·L^-1	1.1(0.6~2.0)	3.5(2.0~4.4)	Z=5.30	<0.001
ANC-Min[M(P₂₅~P₇₅)]/×10⁹·L^-1	0.3(0.1~1.1)	1.1(0.6~2.9)	Z=4.49	<0.001
ANC减少持续时间[M(P₂₅~P₇₅)]/d	4.5(0~10.5)	0(0~2.0)	Z=4.44	<0.001
HB-Min[M(P₂₅~P₇₅)]/g·L^-1	61.5(55.5~74.5)	69.0(63.0~78.0)	Z=2.36	0.018
HB减少持续时间[M(P₂₅~P₇₅)]/d	5.5(2.0~12.0)	8.0(5.0~18.0)	Z=2.30	0.021
PLT-Min[M(P₂₅~P₇₅)]/×10⁹·L^-1	18.0(9.0~47.5)	44.0(12.0~101.0)	Z=2.38	0.017
PLT减少持续时间[M(P₂₅~P₇₅)]/d	6.0(3.0~12.5)	4.0(2.0~9.0)	Z=1.59	0.111
脏器功能指标
TBIL-Max[M(P₂₅~P₇₅)]/μmol·L^-1	49.6(18.0~97.4)	31.9(17.0~84.9)	Z=0.62	0.533
ALT-Max[M(P₂₅~P₇₅)]/U·L^-1	228.5(126.5~441.5)	153.5(57.0~451.0)	Z=1.30	0.194
AST-Max[M(P₂₅~P₇₅)]/U·L^-1	452.0(184.5~781.0)	248.5(103.0~471.0)	Z=1.56	0.118
TG-Max[M(P₂₅~P₇₅)]/mmol·L^-1	3.5(2.9~4.1)	1.9(1.3~3.6)	Z=3.65	<0.001
ALB-Min(x±s)/g·L^-1	26.1±6.4	26.8±7.3	t=0.48	0.632
CREA-Max[M(P₂₅~P₇₅)]/μmol·L^-1	28.0(21.0~39.5)	51.5(33.0~72.0)	Z=4.19	<0.001
凝血指标[M(P₂₅~P₇₅)]
APTT-Max/s	50.8(41.2~63.2)	54.0(44.0~107.6)	Z=1.13	0.257
PT-Max/s	15.2(13.2~22.1)	19.3(14.3~23.6)	Z=1.51	0.130
FIB-Min/g·L^-1	0.9(0.6~1.3)	1.7(1.0~2.4)	Z=3.82	<0.001
D二聚体-Max/mg·L^-1	5.1(2.1~11.4)	3.0(1.0~6.0)	Z=1.41	0.158
炎症指标[M(P₂₅~P₇₅)]
CRP-Max/mg·L^-1	32.5(11.0~101.0)	144.5(81.0~200.0)	Z=4.23	<0.001
PCT-Max/ng·mL^-1	1.1(0.4~4.1)	8.7(4.1~84.8)	Z=5.39	<0.001
SF-Max/ng·mL^-1	4 500.0(1 589.4~4 500.0)	1 133.7(391.8~4 500.0)	Z=3.80	<0.001
LDH-Max/U·L^-1	2 738.0(1 732.5~5 297.5)	2 126.0(1 141.0~5 140.0)	Z=1.47	0.143

变量	偏回归系数	标准误	χ²值	P	OR(95%CI)
WBC-Min	-0.84	0.29	8.66	0.003	0.43(0.25~0.76)
CREA-Max	-0.02	0.01	4.86	0.028	0.98(0.96~0.998)
FIB-Min	-1.11	0.47	5.53	0.019	0.33(0.13~0.83)
PCT-Max	-0.08	0.03	6.43	0.011	0.92(0.87~0.98)
常量	6.31	1.49	17.87	<0.001	－

指标	AUC(95%CI)	P	截断值	灵敏度/%	特异度/%
SF-Max/ng·mL^-1	0.72 (0.62~0.80)	<0.001	1 739.60	73.6	67.6
联合指标	0.95(0.89~0.99)	<0.001	0.77	87.0	90.9

Comparison of clinical features between hemophagocytic lymphohistiocytosis and sepsis

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