Abstract:
BACKGROUND:HELLP (Hemolysis, elevated liver enzymes and low platelets) syndrome is a severe and acute pregnancy-related disorder that occurs in approximately 2.5 per 1000 deliveries and represents a major cause of maternal and perinatal morbidity and mortality. This syndrome has been suggested to be a microangiopathy and delivery is the only effective treatment. OBJECTIVES:The aim of this study was to investigate the pathophysiology of HELLP syndrome by simultaneously exploring complement, haemostasis, autoimmunity and inflammation in relation to the clinical outcome. METHODS:We investigated 19 HELLP patients at the time of diagnosis and 3 months after delivery, for complement function, haemostasis and inflammation with immunoenzymatic methods. Complement-related gene variants were also analyzed by next generation sequencing and multiplex ligation-dependent probe amplification. Nineteen age-matched healthy pregnant women served as controls. RESULTS:At diagnosis, HELLP patients, compared to controls, showed significantly higher plasma levels of SC5b-9 (median 710 ng/ml [range 216-1499] vs 253 ng/ml [19-371], P < 0.0001) and of C5a (20.8 ng/ml [5.6-27.5] vs 12.7 ng/ml [3.2-24.6]; P = 0.004), which decreased three months after delivery (SC5b9: 190 ng/ml [83-446] vs 160 ng/ml [107-219]; C5a: 9.28 ng/ml [2.3-21.6] vs 10.7 ng/ml [2.5-21.2]). A significantly higher frequency of genetic variants involving complement regulatory genes was also observed (52.6% vs 15.8%; P = 0.016). Moreover, at HELLP diagnosis, patients showed increased coagulation markers (fragment F1 + 2 and D-dimer; P = 0.0001) while both patients and controls had high thrombin-generation potential that decreased after delivery. CONCLUSIONS:In the pathophysiology of HELLP syndrome, complement dysregulation, in addition to coagulation activation, is involved and may represent a potential target for treatment with the aim of delaying delivery.
journal_name
Thromb Resjournal_title
Thrombosis researchauthors
Bazzan M,Todros T,Tedeschi S,Ardissino G,Cardaropoli S,Stella S,Montaruli B,Marchese C,Roccatello D,Cugno Mdoi
10.1016/j.thromres.2020.08.038subject
Has Abstractpub_date
2020-12-01 00:00:00pages
167-174eissn
0049-3848issn
1879-2472pii
S0049-3848(20)30486-2journal_volume
196pub_type
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journal_title:Thrombosis research
pub_type: 杂志文章,meta分析,评审
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journal_title:Thrombosis research
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journal_title:Thrombosis research
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doi:10.1016/j.thromres.2003.12.005
更新日期:2003-01-01 00:00:00
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journal_title:Thrombosis research
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pub_type: 临床试验,杂志文章
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journal_title:Thrombosis research
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doi:10.1016/0049-3848(84)90114-2
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doi:10.1016/j.thromres.2013.03.015
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doi:10.1016/0049-3848(90)90335-a
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journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(83)90181-0
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pub_type:
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更新日期:2008-01-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2007-01-01 00:00:00
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journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(84)90195-6
更新日期:1984-12-15 00:00:00
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journal_title:Thrombosis research
pub_type: 杂志文章
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更新日期:2020-12-01 00:00:00
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pub_type: 杂志文章
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journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(83)90238-4
更新日期:1983-04-15 00:00:00
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journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/j.thromres.2020.01.022
更新日期:2020-03-01 00:00:00
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journal_title:Thrombosis research
pub_type: 杂志文章
doi:10.1016/0049-3848(91)90672-j
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