Current insights into thrombotic microangiopathies: Thrombotic thrombocytopenic purpura and pregnancy.

Abstract:

:The complex relation between thrombotic thrombocytopenic purpura (TTP) and pregnancy is concisely reviewed. Pregnancy is a very strong trigger for acute disease manifestation in patients with hereditary TTP caused by double heterozygous or homozygous mutations of ADAMTS13 (ADisintegrin And Metalloprotease with ThromboSpondin type 1 domains, no. 13). In several affected women disease onset during their first pregnancy leads to the diagnosis of hereditary TTP. Without plasma treatment mother and especially fetus are at high risk of dying. The relapse risk during a next pregnancy is almost 100% but regular plasma transfusion starting in early pregnancy will prevent acute TTP flare-up and may result in successful pregnancy outcome. Pregnancy may also constitute a mild risk factor for the onset of acute acquired TTP caused by autoantibody-mediated severe ADAMTS13 deficiency. Women having survived acute acquired TTP may not be at very high risk of TTP relapse during an ensuing next pregnancy but seem to have an elevated risk of preeclampsia. Monitoring of ADAMTS13 activity and inhibitor titre during pregnancy may help to guide management and to avoid disease recurrence. Finally, TTP needs to be distinguished from the much more frequent hypertensive pregnancy complications, preeclampsia and especially HELLP (Hemolysis, Elevated Liver Enzymes, Low Platelet count) syndrome.

journal_name

Thromb Res

journal_title

Thrombosis research

authors

von Auer C,von Krogh AS,Kremer Hovinga JA,Lämmle B

doi

10.1016/S0049-3848(15)50437-4

subject

Has Abstract

pub_date

2015-02-01 00:00:00

pages

S30-3

eissn

0049-3848

issn

1879-2472

pii

S0049-3848(15)50437-4

journal_volume

135 Suppl 1

pub_type

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