Objective To investigate the efficacy and application value of plasma exchange as an adjuvant therapy in children with hemophagocytic syndrome (HPS). Methods A prospective randomized controlled trial was designed. Forty children with severe HPS were enrolled, who were treated in the pediatric intensive care unit (PICU) of Hunan Children's Hospital from October 2018 to October 2020. The children were randomly divided into a plasma exchange group and a conventional treatment group using a random number table, with 20 children in each group. The children in the conventional treatment group received etiological treatment and conventional symptomatic supportive treatment, and those in the plasma exchange group received plasma exchange in addition to the treatment in the conventional treatment group. The two groups were compared in terms of general information, clinical symptoms and signs before and after treatment, main laboratory markers, treatment outcome, and prognosis. Results Before treatment, there were no significant differences between the two groups in gender, age, course of the disease before admission, etiological composition, pediatric critical illness score, involvement of organ or system functions, and laboratory markers (P>0.05). After 7 days of treatment, both groups had remission and improvement in clinical symptoms and signs. After treatment, the plasma exchange group had significantly lower levels of C-reactive protein, procalcitonin, and serum protein levels than the conventional treatment group (P<0.05). The plasma exchange group also had significantly lower levels of alanine aminotransferase and total bilirubin than the conventional treatment group (P<0.05). The length of stay in the PICU in the plasma exchange group was significantly shorter than that in the conventional treatment group (P<0.05). The plasma exchange group had a significantly higher treatment response rate than the conventional treatment group (P<0.05). There were no significant differences between the two groups in the total length of hospital stay and 3-month mortality rate (P>0.05). Conclusions Plasma exchange as an adjuvant therapy is effective for children with severe HPS. It can improve clinical symptoms and signs and some laboratory markers and shorten the length of stay in the PICU, and therefore, it may become an optional adjuvant therapy for children with severe HPS.
Key words
Hemophagocytic syndrome /
Plasma exchange /
Multiple organ dysfunction syndrome /
Child
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References
1 中华医学会儿科学分会血液学组. 噬血细胞性淋巴组织细胞增生症诊疗建议[J]. 中华儿科杂志, 2012, 50(11): 821-825. DOI: 10.3760/cma.j.issn.0578-1310.2012.11.005.
2 Busund R, Koukline V, Utrobin U, et al. Plasmapheresis in severe sepsis and septic shock: a prospective, randomised, controlled trial[J]. Intensive Care Med, 2002, 28(10): 1434-1439. PMID: 12373468. DOI: 10.1007/s00134-002-1410-7.
3 张义刚, 肖政辉. 血浆置换联合连续性血液净化治疗儿童噬血细胞综合征的疗效分析[J]. 实用休克杂志(中英文), 2018, 2(4): 224-229.
4 噬血细胞综合征中国专家联盟, 中华医学会儿科学分会血液学组. 噬血细胞综合征诊治中国专家共识[J]. 中华医学杂志, 2018, 98(2): 91-95. DOI: 10.3760/cma.j.issn.0376-2491.2018.02.004.
5 Goldstein B, Giroir B, Randolph A, et al. International pediatric sepsis consensus conference: definitions for sepsis and organ dysfunction in pediatrics[J]. Pediatr Crit Care Med, 2005, 6(1): 2-8. PMID: 15636651. DOI: 10.1097/01.PCC.0000149131.72248.E6.
6 中国医师协会儿科医师分会血液净化专业委员会. 儿童血浆置换临床应用专家共识[J]. 中华实用儿科临床杂志, 2018, 33(15): 1128-1135. DOI: 10.3760/cma.j.issn.2095-428X.2018.15.003.
7 杨海霞, 郑敏翠, 李婉丽, 等. 儿童噬血细胞综合征临床特征与治疗及预后[J]. 医学临床研究, 2017, 34(7): 1397-1399. DOI: 10.3969/j.issn.1671-7171.2017.07.052.
8 Henter JI, Horne A, Aricó M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis[J]. Pediatr Blood Cancer, 2007, 48(2): 124-131. PMID: 16937360. DOI: 10.1002/pbc.21039.
9 Ansuini V, Rigante D, Esposito S. Debate around infection-dependent hemophagocytic syndrome in paediatrics[J]. BMC Infect Dis, 2013, 13: 15. PMID: 23324497. PMCID: PMC3549728. DOI: 10.1186/1471-2334-13-15.
10 Risma K, Jordan MB. Hemophagocytic lymphohistiocytosis: updates and evolving concepts[J]. Curr Opin Pediatr, 2012, 24(1): 9-15. PMID: 22189397. DOI: 10.1097/MOP.0b013e32834ec9c1.
11 Henter JI, Aricò M, Egeler RM, et al. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. HLH study Group of the Histiocyte Society[J]. Med Pediatr Oncol, 1997, 28(5): 342-347. PMID: 9121398. DOI: 10.1002/(sici)1096-911x(199705)28:5<342::aid-mpo3>3.0.co;2-h.
12 Henter JI, Samuelsson-Horne A, Aricò M, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation[J]. Blood, 2002, 100(7): 2367-2373. PMID: 12239144. DOI: 10.1182/blood-2002-01-0172.
13 张育才, 戎群芳, 陈容欣, 等. 连续性血液净化救治小儿重症噬血细胞综合征[J]. 中华急诊医学杂志, 2013, 22(7): 749-754. DOI: 10.3760/cma.j.issn.1671-0282.2013.07.013.
14 Bosnak M, Erdogan S, Aktekin EH, et al. Therapeutic plasma exchange in primary hemophagocytic lymphohistiocytosis: reports of two cases and a review of the literature[J]. Transfus Apher Sci, 2016, 55(3): 353-356. PMID: 27743708. DOI: 10.1016/j.transci.2016.09.015.
15 Schwartz J, Padmanabhan A, Aqui N, et al. Guidelines on the use of therapeutic apheresis in clinical practice-evidence-based approach from the Writing Committee of the American Society for Apheresis: the seventh special issue[J]. J Clin Apher, 2016, 31(3): 149-162. PMID: 27322218. DOI: 10.1002/jca.21470.
16 中国医师协会儿科医师分会血液净化专业委员会. 噬血细胞综合征患儿血液净化治疗现况调查[J]. 中国小儿急救医学, 2018, 25(8): 607-612. DOI: 10.3760/cma.j.issn.1673-4912.2018.08.012.
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