title: Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes.
abstract: Pregnancy can precipitate thrombotic thrombocytopenic purpura (TTP). We present a prospective study of TTP cases from the United Kingdom Thrombotic Thrombocytopenic Purpura (UK TTP) Registry with clinical and laboratory data from the largest cohort of pregnancy-associated TTP and describe management through pregnancy, averting fetal loss and maternal complications. Thirty-five women presented with a first TTP episode during pregnancy: 23/47 with their first congenital TTP (cTTP) episode and 12/47 with acute acquired TTP in pregnancy. TTP presented primarily in the third trimester/postpartum, but fetal loss was highest in the second trimester. Fetal loss occurred in 16/38 pregnancies before cTTP was diagnosed, but in none of the 15 subsequent managed pregnancies. Seventeen of 23 congenital cases had a missense mutation, C3178T, within exon 24 (R1060W). There were 8 novel mutations. In acquired TTP presentations, fetal loss occurred in 5/18 pregnancies and 2 terminations because of disease. We also present data on 12 women with a history of nonpregnancy-associated TTP: 18 subsequent pregnancies have been successfully managed, guided by ADAMTS13 levels. cTTP presents more frequently than acquired TTP during pregnancy and must be differentiated by ADAMTS13 analysis. Careful diagnosis, monitoring, and treatment in congenital and acquired TTP have assisted in excellent pregnancy outcomes.
authors.affiliation: University College London Hospitals, London, United Kingdom;
authors.forename: Marie
authors.initials: M
authors.lastname: Scully
authors.affiliation: University College London, London, United Kingdom;
authors.forename: Mari
authors.initials: M
authors.lastname: Thomas
authors.affiliation: University College London, London, United Kingdom;
authors.forename: Mary
authors.initials: M
authors.lastname: Underwood
authors.affiliation: Aberdeen Royal Infirmary, Aberdeen, United Kingdom;
authors.forename: Henry
authors.initials: H
authors.lastname: Watson
authors.affiliation: University College London, London, United Kingdom;
authors.forename: Katherine
authors.initials: K
authors.lastname: Langley
authors.affiliation: University of Westminster, London, United Kingdom;
authors.forename: Raymond S
authors.initials: RS
authors.lastname: Camilleri
authors.affiliation: University Hospitals Bristol National Health Service Foundation Trust, Bristol, United Kingdom;
authors.forename: Amanda
authors.initials: A
authors.lastname: Clark
authors.affiliation: Royal Cornwall Hospital Trust, Cornwall, United Kingdom;
authors.forename: Desmond
authors.initials: D
authors.lastname: Creagh
authors.affiliation: Cardiff and Vale University Hospital, Cardiff, United Kingdom;
authors.forename: Rachel
authors.initials: R
authors.lastname: Rayment
authors.affiliation: Guy's and St. Thomas' National Health Service Foundation Trust, London, United Kingdom;
authors.forename: Vickie
authors.initials: V
authors.lastname: Mcdonald
authors.affiliation: Basingstoke and North Hampshire National Health Service Trusts, Basingstoke, Hampshire, United Kingdom;
authors.forename: Ashok
authors.initials: A
authors.lastname: Roy
authors.affiliation: Kent and Canterbury Hospitals, Canterbury, Kent, United Kingdom;
authors.forename: Gillian
authors.initials: G
authors.lastname: Evans
authors.affiliation: University College London Hospitals, London, United Kingdom;
authors.forename: Siobhan
authors.initials: S
authors.lastname: McGuckin
authors.affiliation: The Rotunda Hospital, Dublin, Ireland;
authors.forename: Fionnuala
authors.initials: F
authors.lastname: Ni Ainle
authors.affiliation: Sheffield Teaching Hospital National Health Service Trust, Sheffield, United Kingdom;
authors.forename: Rhona
authors.initials: R
authors.lastname: Maclean
authors.affiliation: University Hospital Birmingham, Birmingham, United Kingdom; and.
authors.forename: William
authors.initials: W
authors.lastname: Lester
authors.affiliation: Manchester Royal Infirmary, Manchester, United Kingdom.
authors.forename: Michael
authors.initials: M
authors.lastname: Nash
authors.affiliation: University College London Hospitals, London, United Kingdom;
authors.forename: Rosemary
authors.initials: R
authors.lastname: Scott
authors.affiliation: University College London Hospitals, London, United Kingdom;
authors.forename: Patrick
authors.initials: P
authors.lastname: O Brien
mesh_terms: D051722:ADAM Proteins / Q000097:blood / Q000235:genetics / Q000276:immunology; D000071120:ADAMTS13 Protein; D037881:Aborted Fetus; D015331:Cohort Studies; D004252:DNA Mutational Analysis; D005260:Female; D006801:Humans; D010920:Placenta / Q000473:pathology; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000628:therapy*; D011256:Pregnancy Outcome / Q000453:epidemiology; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000628:therapy*
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human