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...

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Main Authors: 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
Format: Article
Language:English
Published: Elsevier 2024-11-01
Series:Research and Practice in Thrombosis and Haemostasis
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Online Access:http://www.sciencedirect.com/science/article/pii/S2475037924002747
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author 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
author_facet 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
author_sort Anna Gurevich-Shapiro
collection DOAJ
description 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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spelling doaj-art-6dfd371bbacb480d9edb000862f93a6e2025-08-20T02:32:22ZengElsevierResearch and Practice in Thrombosis and Haemostasis2475-03792024-11-018810257910.1016/j.rpth.2024.102579Complement-mediated hemolytic uremic syndrome associated with postpartum hemorrhage: case series and systematic review of individual participant dataAnna Gurevich-Shapiro0Sharon Orbach-Zinger1Avi Leader2Galia Stemer3Arnon Wiznitzer4Pierre Singer5Miriam Davidovits6Michael Shapiro7Eva N. Hamulyák8Pia Raanani9Galia Spectre10Department of Systems Immunology, Weizmann Institute of Science, Rehovot, Israel; Division of Hematology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel; Faculty of Medicine, Tel Aviv University, Ramat-Aviv, Israel; Correspondence Anna Gurevich-Shapiro, Ido Amit Lab for Immunogenomics, Weizmann Insitute of Science, Rehovot 7610001, Israel.Faculty of Medicine, Tel Aviv University, Ramat-Aviv, Israel; Division of Anesthesia, Rabin Medical Center, Petah Tikva, IsraelFaculty of Medicine, Tel Aviv University, Ramat-Aviv, Israel; Institute of Hematology, Rabin Medical Center, Petah Tikva, IsraelHematology Unit, Haemek Medical Center, Afula, IsraelFaculty of Medicine, Tel Aviv University, Ramat-Aviv, Israel; Helen Schneider Hospital for Women, Rabin Medical Center, Petah Tikva, IsraelFaculty of Medicine, Tel Aviv University, Ramat-Aviv, Israel; Intensive Care Unit, Rabin Medical Center, Petah Tikva, IsraelFaculty of Medicine, Tel Aviv University, Ramat-Aviv, Israel; Institute of Nephrology, Schneider Children’s Medical Center, Rabin Medical Center, Petah Tikva, IsraelFaculty of Medicine, Tel Aviv University, Ramat-Aviv, Israel; Department of Internal Medicine, Tel Aviv Sourasky Medical Center, Tel Aviv, IsraelDepartment of Internal Medicine, Amsterdam University Medical Center, Amsterdam, The NetherlandsFaculty of Medicine, Tel Aviv University, Ramat-Aviv, Israel; Institute of Hematology, Rabin Medical Center, Petah Tikva, IsraelFaculty of Medicine, Tel Aviv University, Ramat-Aviv, Israel; Institute of Hematology, Rabin Medical Center, Petah Tikva, IsraelBackground: 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.http://www.sciencedirect.com/science/article/pii/S2475037924002747postpartum hemorrhagepregnancyComplement C5 inhibitorsatypical hemolytic uremic syndromethrombotic microangiopathiespregnancy complications
spellingShingle 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
Complement-mediated hemolytic uremic syndrome associated with postpartum hemorrhage: case series and systematic review of individual participant data
Research and Practice in Thrombosis and Haemostasis
postpartum hemorrhage
pregnancy
Complement C5 inhibitors
atypical hemolytic uremic syndrome
thrombotic microangiopathies
pregnancy complications
title Complement-mediated hemolytic uremic syndrome associated with postpartum hemorrhage: case series and systematic review of individual participant data
title_full Complement-mediated hemolytic uremic syndrome associated with postpartum hemorrhage: case series and systematic review of individual participant data
title_fullStr Complement-mediated hemolytic uremic syndrome associated with postpartum hemorrhage: case series and systematic review of individual participant data
title_full_unstemmed Complement-mediated hemolytic uremic syndrome associated with postpartum hemorrhage: case series and systematic review of individual participant data
title_short Complement-mediated hemolytic uremic syndrome associated with postpartum hemorrhage: case series and systematic review of individual participant data
title_sort complement mediated hemolytic uremic syndrome associated with postpartum hemorrhage case series and systematic review of individual participant data
topic postpartum hemorrhage
pregnancy
Complement C5 inhibitors
atypical hemolytic uremic syndrome
thrombotic microangiopathies
pregnancy complications
url http://www.sciencedirect.com/science/article/pii/S2475037924002747
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