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Eculizumab, a terminal complement inhibitor, is often used in pregnant patients, but the drug is not universally available. We therefore present the first systematic review with meta-analysis of outcomes in 190 pregnancies from 135 PNH patients, comparing those receiving eculizumab and those not. We searched PubMed for studies reporting on pregnancy outcomes in PNH. Thirty-five papers were selected for inclusion, the majority of which were case studies or small series. Eculizumab was used in 131 pregnancies. There was one maternal death reported in a woman who did not receive eculizumab. A higher rate of fetal survival was observed in pregnancies where eculizumab was used (82%) than those when it was not (69%). Miscarriage (&lt;\u200924 weeks gestation) occurred twice as often in women not receiving eculizumab. Intrauterine death (\u226524 weeks gestation), maternal thrombosis, bleeding (both antepartum and postpartum), and pre-eclampsia were over-represented in PNH pregnancies but did not significantly differ between treatment groups. Premature birth (&lt;\u200937 weeks\u2019 gestation) was observed in 32% of eculizumab pregnancies and 44% of non-eculizumab pregnancies. We conclude that Eculizumab appears to be safe and benefits pregnant women with PNH.<\/jats:p>","DOI":"10.1007\/s00277-025-06353-7","type":"journal-article","created":{"date-parts":[[2025,4,17]],"date-time":"2025-04-17T04:07:43Z","timestamp":1744862863000},"page":"2517-2525","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Paroxysmal nocturnal haemoglobinuria in pregnancy\u2013a systematic review with meta analysis"],"prefix":"10.1007","volume":"104","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4240-659X","authenticated-orcid":false,"given":"James E.","family":"Manning","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Etienne","family":"Ciantar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Morag","family":"Griffin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard J.","family":"Kelly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,4,17]]},"reference":[{"issue":"1","key":"6353_CR1","doi-asserted-by":"publisher","first-page":"17028","DOI":"10.1038\/nrdp.2017.28","volume":"3","author":"A Hill","year":"2017","unstructured":"Hill A, DeZern AE, Kinoshita T, Brodsky RA (2017) Paroxysmal nocturnal haemoglobinuria. 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MG is on the advisory board of Amgen, Alexion, Novartis and Biocryst, has received lecture fees\/honoraria from Alexion, Sobi and Regeneron, has undertaken consultancy for Regeneron and Biocryst and sat on advisory boards for Sobi.RK has received research funding from Sobi and Novartis, sat on advisory boards for Alexion, Jazz, Novartis and Sobi, received lecture fees\/honoraria from Abbvie, Alexion, Astellas, Jazz, Novartis and Sobi and undertaken consultancy for Otsuka and Sobi.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}]}}