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. 2024 Oct 3;8(8):102579.
doi: 10.1016/j.rpth.2024.102579. eCollection 2024 Nov.

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

Anna Gurevich-Shapiro  1   2   3 , Sharon Orbach-Zinger  3   4 , Avi Leader  3   5 , Galia Stemer  6 , Arnon Wiznitzer  3   7 , Pierre Singer  3   8 , Miriam Davidovits  3   9 , Michael Shapiro  3   10 , Eva N Hamulyák  11 , Pia Raanani  3   5 , Galia Spectre  3   5
Affiliations

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

Anna Gurevich-Shapiro et al. Res Pract Thromb Haemost. .

Abstract

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.

Keywords: Complement C5 inhibitors; Parturition; acute kidney injury; atypical hemolytic uremic syndrome; postpartum hemorrhage; postpartum period; pregnancy; pregnancy complications; thrombotic microangiopathies; treatment outcome.

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Figures

Figure 1
Figure 1
Study selection flowchart. CM-HUS, complement-mediated hemolytic uremic syndrome; TMA, thrombotic microangiopathy; PPH, postpartum hemorrhage.
Figure 2
Figure 2
Patient outcomes according to treatment group.
See this image and copyright information in PMC

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