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Semin Thromb Hemost 2016; 42(07): 774-779
DOI: 10.1055/s-0036-1587683
Original Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Thrombotic Thrombocytopenic Purpura and Atypical Hemolytic Uremic Syndrome Microangiopathy in Pregnancy

Marie Scully
1   Department of Hematology, University College London Hospitals, Cardiometabolic Programme-NIHR UCLH/UCL BRC, London, United Kingdom
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Publication History

Publication Date:
20 September 2016 (online)

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Abstract

Thrombotic thrombocytopenic purpura (TTP) and atypical hemolytic uremic syndrome (aHUS) are thrombotic microangiopathies (TMAs) that can present in pregnancy. The presentation can be with typical microangiopathic features and thrombocytopenia, but there is also a significant risk of in-utero fetal loss. TTP presents most commonly in the third trimester and aHUS in the postpartum period. On presumptive diagnosis, plasma exchange should be started and ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity measured. An activity of < 10% is in keeping with TTP, which may be late-onset congenital TTP or acquired disease: the former will require regular plasma therapy and the latter immunosuppression. In aHUS, eculizumab is the therapy of choice. In future pregnancies, follow-up with a multidisciplinary team including fetomaternal and specialist obstetrics should be undertaken.

Keywords

thrombotic thrombocytopenic purpura - TTP - atypical hemolytic uremic syndrome - aHUS - pregnancy
 

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