Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise

Leslie Skeith, Kelle Hurd, Shruti Chaturvedi, Lorraine Chow, Joshua Nicholas, Adrienne Lee, Daniel Young, Dawn Goodyear, Jennifer Soucie, Louis Girard, Antoine Dufour, Ejaife O. Agbani

Research output: Contribution to journalArticlepeer-review


Background: Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare disorder caused by an inherited genetic deficiency of ADAMTS13 and affects less than one per million individuals. Patients who are diagnosed with TTP during pregnancy are at increased risk of maternal and fetal complications including fetal demise. We present a case of a 32-year-old G3P0 (gravida 3, para 0) who presented at 20 weeks gestation with a new diagnosis of congenital TTP (cTTP) and fetal demise. Methods: We describe the pathophysiology of pregnancy complications in a patient with cTTP using platelet procoagulant membrane dynamics analysis and quantitative proteomic studies, compared to four pregnant patients with gestational hypertension, four pregnant patients with preeclampsia, and four healthy pregnant controls. Results: The cTTP patient had increased P-selectin, tissue factor expression, annexin-V binding on platelets and neutrophils, and localized thrombin generation, suggestive of hypercoagulability. Among 15 proteins that were upregulated, S100A8 and S100A9 were distinctly overexpressed. Conclusions: There is platelet-neutrophil activation and interaction, platelet hypercoagulability, and proinflammation in our case of cTTP with fetal demise.

Original languageEnglish (US)
Article number7115
JournalJournal of Clinical Medicine
Issue number23
StatePublished - Dec 2022


  • congenital
  • fetal demise
  • inflammatory markers
  • platelets
  • procoagulant membrane dynamics
  • thrombosis
  • thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Medicine(all)


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