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Title: Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group.
Austin Authors: Fakhouri, Fadi;Scully, Marie;Provôt, François;Blasco, Miquel;Coppo, Paul;Noris, Marina;Paizis, Kathy ;Kavanagh, David;Pène, Frédéric;Quezada, Sol;Hertig, Alexandre;Kissling, Sébastien;O'Brien, Patrick;Delmas, Yahsou;Alberio, Lorenzo;Winer, Norbert;Veyradier, Agnès;Cataland, Spero;Frémeaux-Bacchi, Véronique;Loirat, Chantal;Remuzzi, Giuseppe;Tsatsaris, Vassilis
Affiliation: Service of Nephrology and Hypertension, Department of Medicine, Lausanne University Hospital, Lausanne, Switzerland
Istituto di Ricerche Farmacologiche Mario Negri, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Bergamo, Italy
Complement Therapeutics Research Group, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, United Kingdom
National Renal Complement Therapeutics Centre, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom
Department of Hematology, Hôpital Saint Antoine, Sorbonne Université/AP-HP, Paris, France
Department of Nephrology, Hôpital Huriez, Centre Hospitalier Régional Universitaire (CHRU) de Lille, Lille, France
French Reference Center for Thrombotic Microangiopathies, Hôpital Saint Antoine, Sorbonne Université/Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France
Department of Haematology and
Cardiometabolic Programme, University College London (UCL) Hospitals (UCLH), Biomedical Research Centre (BRC), National Institute for Health Research, National Health Service (NHS) Foundation Trust, London, United Kingdom
Mercy Hospital for Women, Melbourne, VIC, Australia
Sunshine Hospital, Western Health, Melbourne, VIC, Australia
Austin Health
French Reference Center for Thrombotic Microangiopathies, Hôpital Saint Antoine, Sorbonne Université/Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France
Service d'Hématologie Biologique and
Equipe d'accueil3518, Université Paris Diderot, Paris, France
Groupe Hospitalier Saint Louis-Lariboisière, AP-HP, Paris, France
Department of Gynecology and Obstetrics and
Clinical Investigation Center (CIC), University Hospital of Nantes, Nantes, France
Nouvelle Université à Nantes, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement (INRAE), Nantes, France
Physiologie des Adaptations Nutritionnelles (PhAN), UMR 1280, Université de Nantes, Nantes, France
Department of Nephrology, Centre de Référence Maladies Rénales Rares du Sud-ouest, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France
Fetal Medicine Unit-Salud Materno Infantil y del Desarrollo (SAMID), Department of Obstetrics and Gynaecology and
12 de Octubre Research Institute (imas12), University Hospital 12 de Octubre, Complutense University of Madrid, Madrid, Spain
Medical Intensive Care Unit, Cochin Hospital, Hôpitaux Universitaires Paris Centre, AP-HP, Paris, France
Institut Cochin, INSERM Unité 1016 (U1016)/Centre National de la Recherche Scientifique (CNRS) Unité Mixte de Recherche (UMR) 8104/Université de Paris, Paris, France
Department of Nephrology and Renal Transplantation, Hospital Clinic de Barcelona, Universitat de Barcelona, Barcelona, Spain
Department of Kidney Transplantation, Hôpital Pitié-Salpêtrière, Sorbonne Université/AP-HP, Paris, France
Service of Nephrology and Hypertension, Department of Medicine, Lausanne University Hospital, Lausanne, Switzerland
Institute for Women's Health, UCLH, London, United Kingdom
Service and Central Laboratory of Hematology, Lausanne University Hospital, Lausanne, Switzerland
Division of Hematology, The Ohio State University, Columbus, OH
Service d'Immunologie, Hôpital Européen Georges Pompidou, Paris University/AP-HP, Paris, France
Department of Nephrology, University Hospital Robert Debré, Paris, France; and
Maternité Port-Royal, Hôpital Cochin, Université de Paris/AP-HP, Fighting Prematurity University Hospital Federation (FHU PREMA), INSERM UMR 1139, Paris, France
Issue Date: Nov-2020
Publication information: Blood 2020; 136(19): 2103-2117
Abstract: Pregnancy and postpartum are high-risk periods for different forms of thrombotic microangiopathy (TMA). However, the management of pregnancy-associated TMA remains ill defined. This report, by an international multidisciplinary working group of obstetricians, nephrologists, hematologists, intensivists, neonatologists, and complement biologists, summarizes the current knowledge of these potentially severe disorders and proposes a practical clinical approach to diagnose and manage an episode of pregnancy-associated TMA. This approach takes into account the timing of TMA in pregnancy or postpartum, coexisting symptoms, first-line laboratory workup, and probability-based assessment of possible causes of pregnancy-associated TMA. Its aims are: to rule thrombotic thrombocytopenic purpura (TTP) in or out, with urgency, using ADAMTS13 activity testing; to consider alternative disorders with features of TMA (preeclampsia/eclampsia; hemolysis elevated liver enzymes low platelets syndrome; antiphospholipid syndrome); or, ultimately, to diagnose complement-mediated atypical hemolytic uremic syndrome (aHUS; a diagnosis of exclusion). Although they are rare, diagnosing TTP and aHUS associated with pregnancy, and postpartum, is paramount as both require urgent specific treatment.
DOI: 10.1182/blood.2020005221
Journal: Blood
PubMed URL: 32808006
Type: Journal Article
Appears in Collections:Journal articles

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