{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T16:55:47Z","timestamp":1790700947874,"version":"4.1.0"},"reference-count":32,"publisher":"American Society of Hematology","issue":"1","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,12,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Thrombotic microangiopathy (TMA) is the broad definition for thrombocytopenia, microangiopathic hemolytic anemia, and end-organ damage. Two important categories are thrombotic thrombocytopenic purpura (TTP) and complement-mediated hemolytic-uremic syndrome (CM-HUS). Pregnancy and the immediate postpartum period are associated with TMAs specific to pregnancy in rare situations. These include pregnancy-induced hypertension, preeclampsia, and hemolysis, elevated liver enzymes, and low platelets. TTP and CM-HUS may present in pregnancy. However, the diagnosis may not be immediately obvious as they share characteristics of pregnancy-related TMAs. Within this review, we discuss investigations, differential diagnosis of TMAs in pregnancy, and management. The importance is a risk of maternal mortality but also poor fetal outcomes in relation to TTP and CM-HUS. Treatment of these disorders at presentation in pregnancy is discussed to achieve remission and prolong fetal viability if possible. In subsequent pregnancies, a treatment pathway is presented that has been associated with successful maternal and fetal outcomes. Critical to this is a multidisciplinary approach involving obstetricians, the fetal medicine unit, and neonatologists.<\/jats:p>","DOI":"10.1182\/hematology.2021000290","type":"journal-article","created":{"date-parts":[[2021,12,10]],"date-time":"2021-12-10T12:18:16Z","timestamp":1639138696000},"page":"545-551","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":20,"title":["How to evaluate and treat the spectrum of TMA syndromes in pregnancy"],"prefix":"10.1182","volume":"2021","author":[{"given":"Marie","family":"Scully","sequence":"first","affiliation":[{"name":"Department of Haematology, University College London Hospitals NHS Foundation Trust and Cardiometabolic Programme-NIHR UCLH\/UC BRC, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","published-online":{"date-parts":[[2021,12,10]]},"reference":[{"issue":"2","key":"2021121012082739100_hem2021000290C1","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1111\/jth.13571","article-title":"Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies","volume":"15","author":"Scully","year":"2017","journal-title":"J Thromb Haemost"},{"issue":"1","key":"2021121012082739100_hem2021000290C2","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1097\/GRF.0000000000000494","article-title":"Acute fatty liver of pregnancy","volume":"63","author":"Nelson","year":"2020","journal-title":"Clin Obstet Gynecol"},{"issue":"6","key":"2021121012082739100_hem2021000290C3","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1016\/j.ajog.2011.09.002","article-title":"Etiology and management of postpartum hypertension- preeclampsia","volume":"206","author":"Sibai","year":"2012","journal-title":"Am J Obstet Gynecol"},{"issue":"4","key":"2021121012082739100_hem2021000290C4","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1016\/j.tmrv.2018.08.002","article-title":"Microangiopathic hemolytic anemia in pregnancy","volume":"32","author":"Neave","year":"2018","journal-title":"Transfus Med Rev"},{"issue":"6","key":"2021121012082739100_hem2021000290C5","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1111\/bjh.12718","article-title":"How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome","volume":"164","author":"Scully","year":"2014","journal-title":"Br J Haematol"},{"issue":"6","key":"2021121012082739100_hem2021000290C6","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1111\/bjh.14045","article-title":"How we manage thrombotic microangiopathies in pregnancy","volume":"173","author":"Thomas","year":"2016","journal-title":"Br J Haematol"},{"issue":"6","key":"2021121012082739100_hem2021000290C7","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1002\/uog.17455","article-title":"Multicenter screening for pre-eclampsia by maternal factors and biomarkers at 11-13 weeks' gestation: comparison with NICE guidelines and ACOG recommendations","volume":"49","author":"O'Gorman","year":"2017","journal-title":"Ultrasound Obstet Gynecol"},{"issue":"3","key":"2021121012082739100_hem2021000290C8","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1038\/hr.2016.126","article-title":"Epidemiology of hypertensive disorders in pregnancy: prevalence, risk factors, predictors and prognosis","volume":"40","author":"Umesawa","year":"2017","journal-title":"Hypertens Res"},{"issue":"7","key":"2021121012082739100_hem2021000290C9","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1055\/s-0036-1587683","article-title":"Thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome microangiopathy in pregnancy","volume":"42","author":"Scully","year":"2016","journal-title":"Semin Thromb Hemost"},{"issue":"1","key":"2021121012082739100_hem2021000290C10","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1007\/s00431-008-0696-z","article-title":"Acute fatty liver of pregnancy and neonatal long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency","volume":"168","author":"Guti\u00e9rrez Junquera","year":"2009","journal-title":"Eur J Pediatr"},{"issue":"1","key":"2021121012082739100_hem2021000290C11","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1182\/blood-2012-08-448944","article-title":"How I treat thrombocytopenia in pregnancy","volume":"121","author":"Gernsheimer","year":"2013","journal-title":"Blood"},{"issue":"3","key":"2021121012082739100_hem2021000290C12","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1080\/09537104.2019.1640870","article-title":"Managing ITP and thrombocytopenia in pregnancy","volume":"31","author":"Eslick","year":"2020","journal-title":"Platelets"},{"issue":"suppl","key":"2021121012082739100_hem2021000290C13","first-page":"1","article-title":"KDIGO clinical practice guideline for acute kidney injury","volume":"2","author":"Kidney Disease: Improving Global Outcomes (KDIGO) Acute Kidney Injury Work Group","year":"2012","journal-title":"Kidney Int"},{"issue":"9","key":"2021121012082739100_hem2021000290C14","doi-asserted-by":"crossref","first-page":"2730","DOI":"10.1182\/blood.V98.9.2730","article-title":"Changes in health and disease of the metalloprotease that cleaves von Willebrand factor","volume":"98","author":"Mannucci","year":"2001","journal-title":"Blood"},{"issue":"6","key":"2021121012082739100_hem2021000290C15","first-page":"1320","article-title":"von Willebrand factor-cleaving protease (ADAMTS13) activity in normal non-pregnant women, pregnant and post-delivery women","volume":"92","author":"S\u00e1nchez-Luceros","year":"2004","journal-title":"Thromb Haemost"},{"issue":"6","key":"2021121012082739100_hem2021000290C16","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1007\/s13224-011-0093-9","article-title":"Lactic dehydrogenase: a biochemical marker for preeclampsia-eclampsia","volume":"61","author":"Jaiswar","year":"2011","journal-title":"J Obstet Gynaecol India"},{"issue":"2","key":"2021121012082739100_hem2021000290C17","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1182\/blood-2014-02-553131","article-title":"Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes","volume":"124","author":"Scully","year":"2014","journal-title":"Blood"},{"issue":"24","key":"2021121012082739100_hem2021000290C18","doi-asserted-by":"crossref","first-page":"5888","DOI":"10.1182\/blood-2012-02-408914","article-title":"Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura","volume":"119","author":"Moatti-Cohen","year":"2012","journal-title":"Blood"},{"issue":"1","key":"2021121012082739100_hem2021000290C19","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1016\/j.molimm.2015.02.030","article-title":"The complement system and adverse pregnancy outcomes","volume":"67","author":"Regal","year":"2015","journal-title":"Mol Immunol"},{"issue":"5","key":"2021121012082739100_hem2021000290C20","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1016\/j.exphem.2016.01.005","article-title":"Direct evidence of complement activation in HELLP syndrome: a link to atypical hemolytic uremic syndrome","volume":"44","author":"Vaught","year":"2016","journal-title":"Exp Hematol"},{"issue":"3","key":"2021121012082739100_hem2021000290C21","doi-asserted-by":"crossref","first-page":"e1001013","DOI":"10.1371\/journal.pmed.1001013","article-title":"Mutations in complement regulatory proteins predispose to preeclampsia: a genetic analysis of the PROMISSE cohort","volume":"8","author":"Salmon","year":"2011","journal-title":"PLoS Med"},{"issue":"3","key":"2021121012082739100_hem2021000290C22","doi-asserted-by":"crossref","first-page":"1020","DOI":"10.1681\/ASN.2016090995","article-title":"Maternal and fetal outcomes of pregnancies in women with atypical hemolytic uremic syndrome","volume":"29","author":"Gaggl","year":"2018","journal-title":"J Am Soc Nephrol"},{"issue":"3","key":"2021121012082739100_hem2021000290C23","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1080\/10641955.2019.1640725","article-title":"Placental sFLT1 is associated with complement activation and syncytiotrophoblast damage in preeclampsia","volume":"38","author":"Yonekura Collier","year":"2019","journal-title":"Hypertens Pregnancy"},{"issue":"15","key":"2021121012082739100_hem2021000290C24","doi-asserted-by":"crossref","first-page":"1644","DOI":"10.1182\/blood-2018-11-884700","article-title":"Characterization and treatment of congenital thrombotic thrombocytopenic purpura","volume":"133","author":"Alwan","year":"2019","journal-title":"Blood"},{"issue":"4","key":"2021121012082739100_hem2021000290C25","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1056\/NEJMoa1806311","article-title":"Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura","volume":"380","author":"Scully","year":"2019","journal-title":"N Engl J Med"},{"issue":"6","key":"2021121012082739100_hem2021000290C26","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1007\/s12185-016-1946-x","article-title":"Pregnancy outcomes of patients with paroxysmal nocturnal hemoglobinuria treated with eculizumab: a Japanese experience and updated review","volume":"103","author":"Miyasaka","year":"2016","journal-title":"Int J Hematol"},{"issue":"11","key":"2021121012082739100_hem2021000290C27","doi-asserted-by":"crossref","first-page":"1032","DOI":"10.1056\/NEJMoa1502950","article-title":"Eculizumab in pregnant patients with paroxysmal nocturnal hemoglobinuria","volume":"373","author":"Kelly","year":"2015","journal-title":"N Engl J Med"},{"issue":"12","key":"2021121012082739100_hem2021000290C28","doi-asserted-by":"crossref","first-page":"2122","DOI":"10.1093\/ndt\/gfw314","article-title":"Atypical haemolytic uraemic syndrome and pregnancy: outcome with ongoing eculizumab","volume":"31","author":"Servais","year":"2016","journal-title":"Nephrol Dial Transplant"},{"key":"2021121012082739100_hem2021000290C29","doi-asserted-by":"crossref","DOI":"10.1007\/s40620-021-01025-x","article-title":"Pregnancy-triggered atypical hemolytic uremic syndrome (aHUS): a Global aHUS Registry analysis [published online 7 April 2021]","author":"Fakhouri","year":"2021","journal-title":"J Nephrol"},{"issue":"7","key":"2021121012082739100_hem2021000290C30","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1002\/pd.4403","article-title":"Low-dose aspirin for prevention of adverse outcomes related to abnormal placentation","volume":"34","author":"Bujold","year":"2014","journal-title":"Prenat Diagn"},{"issue":"19","key":"2021121012082739100_hem2021000290C31","doi-asserted-by":"crossref","first-page":"2103","DOI":"10.1182\/blood.2020005221","article-title":"Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group","volume":"136","author":"Fakhouri","year":"2020","journal-title":"Blood"},{"issue":"11","key":"2021121012082739100_hem2021000290C32","doi-asserted-by":"crossref","first-page":"2929","DOI":"10.1111\/jth.15064","article-title":"Success and limitations of plasma treatment in pregnant women with congenital thrombotic thrombocytopenic purpura","volume":"18","author":"Sakai","year":"2020","journal-title":"J Thromb Haemost"}],"container-title":["Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/ashpublications.org\/hematology\/article-pdf\/2021\/1\/545\/1851434\/545scully.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/ashpublications.org\/hematology\/article-pdf\/2021\/1\/545\/1851434\/545scully.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,10]],"date-time":"2021-12-10T12:18:49Z","timestamp":1639138729000},"score":1,"resource":{"primary":{"URL":"https:\/\/ashpublications.org\/hematology\/article\/2021\/1\/545\/483001\/How-to-evaluate-and-treat-the-spectrum-of-TMA"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12,10]]},"references-count":32,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021,12,10]]},"published-print":{"date-parts":[[2021,12,10]]}},"URL":"https:\/\/doi.org\/10.1182\/hematology.2021000290","relation":{},"ISSN":["1520-4391","1520-4383"],"issn-type":[{"value":"1520-4391","type":"print"},{"value":"1520-4383","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2021,12,10]]},"published":{"date-parts":[[2021,12,10]]}}}