妊娠引起的噬血细胞性淋巴组织细胞增生症：病例报告与文献回顾

doi:10.24920/003603

摘要/Abstract

摘要：

噬血细胞性淋巴组织细胞增生症（hemophagocytic lymphohistiocytosis, HLH）是一种罕见但严重威胁健康的疾病,其典型特征为免疫反应失调和过度的炎症反应。目前关于妊娠引起的HLH鲜有报道。本文报道了一例自怀孕17周以来持续发烧21天的30岁孕妇,通过一系列详尽的检查排除感染、自身免疫性疾病和恶性肿瘤等可能病因,给予广谱抗生素和地塞米松等治疗后,病情仍在进展。患者被诊断为妊娠引起的HLH。终止妊娠并继续使用糖皮质激素控制过度炎症反应后,该患者疾病痊愈。妊娠引起的HLH若不及时给予有效的治疗,会严重威胁生命健康。进一步研究以提高HLH早期诊断和病因鉴定至关重要。

关键词: 噬血细胞作用, 过度炎症, 妊娠

Abstract:

Hemophagocytic lymphohistiocytosis (HLH) is a rare but devastating disease characterized by dysregulated immune response and hyperinflammation. To our knowledge, pregnancy-induced HLH has been rarely reported in the literature. A 30-year-old pregnant woman presented persistent fever for 21 days since 17 weeks of pregnancy. The possible etiologies such as infection, autoimmune disorder, and malignancy had been ruled out based on a series of exhaustive examinations. The disease progressed despite the use of broad-spectrum antibiotics and dexamethasone. The patient was diagnosed as pregnancy-induced HLH, and finally recovered completely after termination of pregnancy by caesarean and the continuous use of glucocorticoid which played a crucial part in controlling hyperinflammation. Pregnancy-induced HLH could be fatal if effective treatment was not initiated timely. Further studies are needed to improve early diagnosis and etiology identification of HLH.

Key words: hemophagocytosis, hyperinflammation, pregnancy

Jie Zhao, Yimeng Yang, Shuhong Ming. Pregnancy-Induced Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review[J].Chinese Medical Sciences Journal, 2020, 35(4): 371-376.

图/表 2

No.	Reference	Maternal age (years)	Gestation (weeks)	Associated disease	Treatment	Maternal outcome	Fetal outcome
1	Nakabayashi et al., 1999^[8]	30	21	Preeclampsia	Antithrombin	survive	survive
2	Chmait et al., 2000^[9]	24	29	Necrotizing lymphadenitis, EBV	Ampicillin, acyclovir Betamethasone, IgG	death	survive
3	Hanaoka et al., 2007^[10]	33	21	B-cell lymphoma	Emergent caesarean section ; R-CHOP chemotherapy	survive	survive
4	Teng et al., 2009^[11]	28	23	Autoimmune haemolytic anaemia	No response to corticosteroid; caesarean section	survive	death
5	Arewa et al., 2011^[12]	31	21	HIV	HAART	survive	survive
6	Shukla et al., 2013^[13]	23	10	NS	No response to prednisolone; spontaneous abortion	survive	death
7	Goulding et al., 2014^[14]	27	23	HSV-2	Acyclovir	survive	death
8	Mayama et al., 2014^[15]	28	20	Parvovirus B19	Prednisolone	survive	survive
9	Klein et al., 2014^[16]	39	30	EBV	Corticosteroid, cyclosporine A, etoposide, Rituximab	death	survive
10	Tumian et al., 2015^[17]	35	38	CMV	Dexamethasone	death	survive
11	Giard et al., 2016^[18]	35	13	Streptococcal pharyngitis, KF lymphadenitis	No response to corticosteroids; dexamethasone and etoposide; abortion	death	death
12	Rousselin et al., 2017^[19]	44	30	Raynaud syndrome, thrombocytopenia	Antimicrobial therapy, glucocorticosteroids	survive	survive
13	He et al., 2017^[20]	27	30	NK/T cells lymphoma	Dexamethasone, rituximab and etoposide	death	survive
14	Ikeda et al., 2017^[21]	36	11	EBV	Dexamethasone, cyclosporine, Etoposide	survive	survive
15	Yildiz et al., 2018^[22]	36	NS	Acute hepatitis, HIV	Metronidazole, ceftriaxone, methylprednisolone, antibiotherapy	survive	survive
16	Wang et al., 2018^[23]	40	NS	UNC13D gene mutation	Etoposide, dexamethasone	survive	death
17	Nasser et al., 2018^[7]	36	31	HSV-2	Acyclovir	survive	death
18	Neistadt et al., 2018^[24]	NS	27	NK/T-cell lymphoma	Corticosteroids, etoposide, pembrolizumab	death	death
19	Parrott et al., 2019^[25]	28	18	Lupus	No response to etoposide and corticosteroid	death	death

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Laboratory test indexes	Admission (2018-9-26)	Rapid progression (2018-10-16)	Recovery (2018-11-6)	Normal range
White blood cell (×10⁹/L)	2.6	3.9	10.0	4-10
Hemoglobin (g/L)	91	85	90	110-150
Platelet (×10⁹/L)	169	49	253	100-300
C-reaction protein (mg/dL)	7.1	17.1	1.4	<0.8
Procalcitonin (ng/mL）	0.72	0.78	<0.05	<0.05
Sodium (mmol/L)	129.6	131.1	137.6	135-148
Lactate dehydrogenase (U/L)	663	1109	189	109-245
Triglyceride (mmol/L)	3.8	4.5	1.8	<1.7
Ferritin (ng/mL)	4378.8	12445.2	453.8	4.63-204
Activity of NK cell (%)	16.4	15.2	/	>15.11
Soluble CD25 (pg/mL)	5267	13170	7137	<64000
D-dimer (ng/mL)	8314	52309	1927	<1000

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