A retrospective study of children with hemophagocytic syndrome

doi:10.3877/cma.j.issn.2095-5820.2021.02.005

Abstract

Abstract:

Objective

To investigate the clinical features, laboratory results, treatment and prognosis of children with hemophagocytic syndrome (HPS) for improving the clinical understanding of the disease

Methods

The clinical data of 17 children with HPS admitted to the pediatric intensive care unit (PICU) in Guangdong Women and Children Hospital from January 2016 to March 2020 were retrospectively analyzed.

Results

The most common clinical symptoms of the 17 children with HPS were fever (100%), hepatomegaly (52.94%) and rash (52.94%), followed by superficial lymphadenopathy (23.53%), tricavitary sign (17.65%), splenomegaly (11.76%) and jaundice (11.76%). In laboratory examination, 14 cases (82.35%) of hemocyte secondary or tertiary lines decreased, 17 cases (100%) of ferritin increased, 16 cases (94.12%) of D-dimer increased, 16 cases (94.12%) of hemoglobin decreased, 15 cases (88.24%) of partially activated thromboplastin time prolonged. Hemophilic phenomenon of bone marrow was observed in all patients. There were 5 cases of autoimmune diseases related antibodies and 7 cases of EB virus positive. After treatment, 12 patients recovered and 5 cases died.

Conclusion

Considering the complex clinical presentation and various etiology of children with HPS, dynamic observation of laboratory results and expansion of diagnostic strategy were needed to achieve the purpose of early diagnosis

Key words: Hemophagocytic syndrome, Children, Clinical feature, Laboratory results

Xin Luo, Yanlong Huang, Huili Wang, Shunyan Duan, Xiaoping Mu. A retrospective study of children with hemophagocytic syndrome[J]. Chinese Journal of Clinical Laboratory Management(Electronic Edition), 2021, 09(02): 84-87.

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Figures/Tables 3

References 12

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检测项目	中位数(P25，P75)	异常率(%)
WBC(×10⁹/L)	3.170(2.100，10.23)	88.24
N(×10⁹/L)	2.120(1.090，5.480)	47.06
RBC(×10¹²/L)	3.310(2.990，3.630)	88.24
HGB(g/L)	88.00(74.50，101.5)	94.12
PLT(×10⁹/L)	66.00(37.50，104.0)	82.35
ALT(U/L)	62.00(39.00，218.0)	58.82
AST(U/L)	194.0(64.50，435.5)	82.35
ALP(U/L)	144.0(65.50，244.0)	41.18
GGT(U/L)	78.50(21.25，146.8)	58.82
TP(g/L)	50.70(46.70，59.13)	64.71
A/G	0.800(0.710，1.100)	88.24
CREA(umol/L)	30.80(24.45，39.85)	29.41
UREA(mmol/L)	2.680(1.890，4.710)	64.71
UA(umol/L)	236.6(160.5，410.4)	64.71
APTT(s)	54.60(40.00，76.60)	88.24
PT(s)	14.40(12.60，16.10)	58.82
TT(s)	24.70(19.40，32.80)	70.59
Fbg(g/L)	1.690(1.220，2.440)	76.47
AT-Ⅲ(%)	57.30(50.70，93.65)	64.71
D-Dimer(mg/L)	6.610(3.160，17.09)	94.12
FER(μg/L)	4 000.0(1045，11908)	100.0
TG(mmol/L)	2.700(1.290，3.660)	41.12

检测项目	中位数(P25，P75)	异常率(%)
WBC(×10⁹/L)	3.170(2.100，10.23)	88.24
N(×10⁹/L)	2.120(1.090，5.480)	47.06
RBC(×10¹²/L)	3.310(2.990，3.630)	88.24
HGB(g/L)	88.00(74.50，101.5)	94.12
PLT(×10⁹/L)	66.00(37.50，104.0)	82.35
ALT(U/L)	62.00(39.00，218.0)	58.82
AST(U/L)	194.0(64.50，435.5)	82.35
ALP(U/L)	144.0(65.50，244.0)	41.18
GGT(U/L)	78.50(21.25，146.8)	58.82
TP(g/L)	50.70(46.70，59.13)	64.71
A/G	0.800(0.710，1.100)	88.24
CREA(umol/L)	30.80(24.45，39.85)	29.41
UREA(mmol/L)	2.680(1.890，4.710)	64.71
UA(umol/L)	236.6(160.5，410.4)	64.71
APTT(s)	54.60(40.00，76.60)	88.24
PT(s)	14.40(12.60，16.10)	58.82
TT(s)	24.70(19.40，32.80)	70.59
Fbg(g/L)	1.690(1.220，2.440)	76.47
AT-Ⅲ(%)	57.30(50.70，93.65)	64.71
D-Dimer(mg/L)	6.610(3.160，17.09)	94.12
FER(μg/L)	4 000.0(1045，11908)	100.0
TG(mmol/L)	2.700(1.290，3.660)	41.12

序号	性别	月龄	血红蛋白 (g/L)	血小板 (/L)	纤维蛋白原 (g/L)	三酰甘油 (mmol/L)	铁蛋白 (μg/L )	自身抗体谱	病原学检测	治疗	转归
1	男	24	81	13×10⁹	0.34	2.19	40 000	阴性	EB病毒	抗感染+激素+VP-16	好转
2	女	36	73	25×10⁹	0.3	4.05	681	阴性	白色念珠菌	抗感染	死亡
3	男	4	66	54×10⁹	1.53	3.43	40 000	阴性	巨细胞病毒	抗感染+激素	好转
4	女	7	76	31×10⁹	1.03	1.49	176	抗SSA/Ro-52，抗SSA/Ro-60	白色念珠菌	抗感染+激素	好转
5	女	48	112	66×10⁹	1.78	0.96	3 895	阴性	金黄色葡萄球菌	抗感染+激素	好转
6	女	48	99	55×10⁹	1.78	2.95	7 553	抗SSA/Ro-60	EB病毒	抗感染+激素+IVIG	好转
7	男	24	104	241×10⁹	3.61	4.06	13 392	阴性	EB病毒	抗感染+激素+VP-16	好转
8	女	48	92	74×10⁹	1.86	1.67	33 708	阴性	嗜麦芽窄食单胞菌	抗感染+激素+VP-16	死亡
9	女	36	90	44×10⁹	1.57	2.7	40 000	阴性	EB病毒	抗感染+激素	好转
10	女	84	105	151×10⁹	1.40	0.31	40 000	阴性	A型流感病毒	抗感染+激素	死亡
11	男	12	67	100×10⁹	1.69	1.82	1 408	阴性	EB病毒	抗感染+激素+IVIG	死亡
12	女	60	96	108×10⁹	2.63	1.03	10 423	阴性	金黄色葡萄球菌	抗感染+激素+VP-16+环孢素	好转
13	男	24	86	85×10⁹	5.42	3.65	609	抗双链DNA抗体	A型流感病毒	抗感染	好转
14	女	9	76	78×10⁹	2.58	3.66	34 966	阴性	A型流感病毒	抗感染+激素+VP-16	好转
15	男	12	69	58×10⁹	1.65	3.44	5 420	抗双链DNA抗体	肺炎支原体	抗感染	好转
16	男	12	88	125×10⁹	2.3	1.08	417	抗双链DNA抗体，抗核提取物抗体	EB病毒	抗感染+激素	好转
17	女	24	106	31×10⁹	0.89	3.75	19 943	阴性	EB病毒	抗感染	死亡

序号	性别	月龄	血红蛋白 (g/L)	血小板 (/L)	纤维蛋白原 (g/L)	三酰甘油 (mmol/L)	铁蛋白 (μg/L )	自身抗体谱	病原学检测	治疗	转归
1	男	24	81	13×10⁹	0.34	2.19	40 000	阴性	EB病毒	抗感染+激素+VP-16	好转
2	女	36	73	25×10⁹	0.3	4.05	681	阴性	白色念珠菌	抗感染	死亡
3	男	4	66	54×10⁹	1.53	3.43	40 000	阴性	巨细胞病毒	抗感染+激素	好转
4	女	7	76	31×10⁹	1.03	1.49	176	抗SSA/Ro-52，抗SSA/Ro-60	白色念珠菌	抗感染+激素	好转
5	女	48	112	66×10⁹	1.78	0.96	3 895	阴性	金黄色葡萄球菌	抗感染+激素	好转
6	女	48	99	55×10⁹	1.78	2.95	7 553	抗SSA/Ro-60	EB病毒	抗感染+激素+IVIG	好转
7	男	24	104	241×10⁹	3.61	4.06	13 392	阴性	EB病毒	抗感染+激素+VP-16	好转
8	女	48	92	74×10⁹	1.86	1.67	33 708	阴性	嗜麦芽窄食单胞菌	抗感染+激素+VP-16	死亡
9	女	36	90	44×10⁹	1.57	2.7	40 000	阴性	EB病毒	抗感染+激素	好转
10	女	84	105	151×10⁹	1.40	0.31	40 000	阴性	A型流感病毒	抗感染+激素	死亡
11	男	12	67	100×10⁹	1.69	1.82	1 408	阴性	EB病毒	抗感染+激素+IVIG	死亡
12	女	60	96	108×10⁹	2.63	1.03	10 423	阴性	金黄色葡萄球菌	抗感染+激素+VP-16+环孢素	好转
13	男	24	86	85×10⁹	5.42	3.65	609	抗双链DNA抗体	A型流感病毒	抗感染	好转
14	女	9	76	78×10⁹	2.58	3.66	34 966	阴性	A型流感病毒	抗感染+激素+VP-16	好转
15	男	12	69	58×10⁹	1.65	3.44	5 420	抗双链DNA抗体	肺炎支原体	抗感染	好转
16	男	12	88	125×10⁹	2.3	1.08	417	抗双链DNA抗体，抗核提取物抗体	EB病毒	抗感染+激素	好转
17	女	24	106	31×10⁹	0.89	3.75	19 943	阴性	EB病毒	抗感染	死亡

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