title: 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.
authors.affiliation: Department of Medicine 3, University Medical Center, Mainz, Germany; Center for Thrombosis and Hemostasis, University Medical Center, Mainz, Germany.
authors.forename: Charis
authors.initials: C
authors.lastname: von Auer
authors.affiliation: Department of Cancer Research and Molecular Medicine, Norwegian University of Science and Technology and Department of Haematology, St. Olavs Hospital, Trondheim University Hospital, Trondheim, Norway.
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: von Krogh
authors.affiliation: Department of Hematology and Central Hematology Laboratory, Inselspital Bern University Hospital and University of Bern, Bern, Switzerland.
authors.forename: Johanna A
authors.initials: JA
authors.lastname: Kremer Hovinga
authors.affiliation: Center for Thrombosis and Hemostasis, University Medical Center, Mainz, Germany; Department of Hematology and Central Hematology Laboratory, Inselspital Bern University Hospital and University of Bern, Bern, Switzerland. Electronic address: Bernhard.laemmle@uni-mainz.de.
authors.forename: Bernhard
authors.initials: B
authors.lastname: Lämmle
mesh_terms: D051722:ADAM Proteins / Q000235:genetics*; D000071120:ADAMTS13 Protein; D015984:Causality; D015897:Comorbidity; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D015994:Incidence; D009154:Mutation / Q000235:genetics; D020641:Polymorphism, Single Nucleotide / Q000235:genetics; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000235:genetics* / Q000401:mortality*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000235:genetics* / Q000401:mortality*; D012307:Risk Factors; D015996:Survival Rate
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: ADAMTS13 activity; Pregnancy; Thrombotic thrombocytopenic purpura (TTP)