补体介导的与产后出血相关的溶血性尿毒症综合征：病例系列和个体参与者数据的系统回顾

IF 3.4 3区医学 Q2 HEMATOLOGY

Research and Practice in Thrombosis and Haemostasis Pub Date : 2024-11-01 DOI:10.1016/j.rpth.2024.102579

Anna Gurevich-Shapiro , Sharon Orbach-Zinger , Avi Leader , Galia Stemer , Arnon Wiznitzer , Pierre Singer , Miriam Davidovits , Michael Shapiro , Eva N. Hamulyák , Pia Raanani , Galia Spectre

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引用次数: 0

摘要

背景：产后出血被认为是妊娠相关补体介导的溶血性尿毒症综合征(CM-HUS；以前称为非典型溶血性尿毒症综合征)，但尚未系统研究。目的：系统探讨产后出血在CM-HUS发病中的作用，探讨产后出血相关CM-HUS的特点、预后及建议的治疗方法。方法：系统回顾来自病例系列和报告的个体参与者数据，以及我们机构的病例系列。搜索词是“血栓性微血管病”、“非典型溶血性尿毒症综合征”和“补体介导”合并“妊娠”、“产后”和/或“产后出血”。排除CM-HUS以外的血栓性微血管病变病例。结果是产后出血相关cm -溶血性尿毒综合征的临床和实验室特征、治疗和结果。结果：共纳入33项研究，包括48名产后出血相关CM-HUS患者和我院3名患者。大多数妇女在足月出现(28/45；62%)，剖宫产分娩(21/41；51%)，并有妊娠并发症，主要为子痫前期(16/51；31%)或胎儿死亡(9/51；18%)。血液学和肾脏异常通常在产后24小时内出现。血小板计数中位数为46 × 109/L (IQR, 26-72)，乳酸脱氢酶中位数最大值为2638 U/L （IQR, 1620-3588）。有20/23（87%）的女性接受C5抑制剂治疗伴或不伴血浆置换后肾功能恢复正常；7/11（63%）的女性单独接受血浆置换治疗，而只有3/17（18%）的患者接受支持性治疗。与单纯支持治疗相比，接受C5抑制剂和/或血浆置换治疗的患者获得了明显更好的肾脏预后（P < 0.001）。结论：CM-HUS是一种罕见的产后出血并发症，主要发生在子痫前期和/或剖宫产术后。与单独接受支持治疗的患者相比，接受C5抑制剂和/或血浆置换治疗的患者具有更好的肾脏预后。

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Complement-mediated hemolytic uremic syndrome associated with postpartum hemorrhage: case series and systematic review of individual participant data

Background

Postpartum hemorrhage is considered a risk factor for pregnancy-associated complement-mediated hemolytic uremic syndrome (CM-HUS; previously known as atypical hemolytic uremic syndrome) but has not been systematically studied.

Objectives

To systematically examine the role of postpartum hemorrhage in precipitating CM-HUS and to describe the characteristics of postpartum hemorrhage-associated CM-HUS, its prognosis and recommended management.

Methods

A systematic review of individual participant data from case series and reports in addition to a case series from our institution. Search terms were “thrombotic microangiopathy,” “atypical hemolytic uremic syndrome,” and “complement mediated” combined with “pregnancy,” “postpartum,” and/or “postpartum hemorrhage”. Cases of thrombotic microangiopathy other than CM-HUS were excluded. Outcomes were clinical and laboratory characteristics of postpartum hemorrhage-associated CM-HUS, treatment, and outcomes.

Results

Thirty-three studies comprising 48 women with postpartum hemorrhage-associated CM-HUS and 3 patients from our institution were included in the study. Most women presented at term (28/45; 62%), delivered by cesarean section (21/41; 51%), and had pregnancy complications, mainly preeclampsia (16/51; 31%) or fetal demise (9/51; 18%). Hematological and renal abnormalities usually appeared within the first 24 hours postdelivery. The median platelet count was 46 × 10⁹/L (IQR, 26-72), and the median maximal lactate dehydrogenase was 2638 U/L (IQR, 1620-3588). Renal function normalized in 20/23 (87%) women treated with C5 inhibitors with or without plasma exchange; in 7/11 (63%) women treated with plasma exchange alone, but only in 3/17 (18%) patients treated with supportive care. Patients treated with C5 inhibitors and/or plasma exchange achieved significantly better renal outcomes compared with supportive care alone (P < .001).

Conclusion

CM-HUS is a rare complication following postpartum hemorrhage and occurs mainly in women with preeclampsia and/or following cesarean section. Patients treated with C5 inhibitors and/or plasma exchange had a better renal prognosis compared with patients who received supportive treatment alone.

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