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Poster Networking

Women's Hemostasis and Thrombosis

PB0475 - Thrombotic Microangiopathy during Pregnancy: The Role of ADAMTS13 Activity

Sunday, June 23, 2024
13:45 – 14:45 ICT
Room: Exhibition Hall

    Presenting Author(s)

  • EG

    Eleni Gavriilaki

    Dr
    2nd Propaedeutic Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

    • Co-Author(s)

  • DB

    Despoina Barbarousi

    Dr
    Hematology Department, Alexandra Hospital, Athens, Greece
    Athens, Attiki, Greece

  • EP

    Emmanuel Papadakis

    Dr
    Hematology Department, Genesis, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • EK

    Eudoxia-Evaggelia Koravou

    Dr
    Hematology Department, BMT Unit, G. Papanicolaou Hospital, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • TT

    Tasoula Touloumenidou

    Dr
    Hematology Department, BMT Unit, G. Papanicolaou Hospital, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • AP

    Apostolia Papalexandri

    Dr
    Hematology Department, BMT Unit, G. Papanicolaou Hospital, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • KB

    Katerina Balaska

    Dr
    2nd Propaedeutic Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • NK

    Nikolaos Kotsiou

    Scientific Associate
    2nd Propaedeutic Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • SC

    Sofia Chissan

    Dr
    2nd Propaedeutic Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • AB

    Anastasia Banti

    Dr
    Hematology Department, Papageorgiou Hospital, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • CM

    Charis Matsouka

    Dr
    Hematology Department, Alexandra Hospital, Athens, Greece
    Athens, Attiki, Greece

  • NR

    Niki Rougkala

    Dr
    6Hematology Department, Sismanogleion Hospital, Athens, Greece
    Athens, Attiki, Greece

  • TL

    Theoni Leonidopoulou

    Dr
    Hematology Department, Sismanogleion Hospital, Athens, Greece
    Athens, Attiki, Greece

  • EM

    Evdokia Mandala

    Dr
    Forth Department of Medicine, Faculty of Medicine, Aristotle University of Thessaloniki, Hippokration Hospital, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • MD

    Michael Doumas

    Director
    2nd Propaedeutic Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • IS

    Ioanna Sakellari

    Director
    Hematology Department, BMT Unit, G. Papanicolaou Hospital, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

  • SV

    Sofia Vakalopoulou

    Dr
    2nd Propaedeutic Department of Internal Medicine, Hippokration General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece
    Thessaloniki, Thessaloniki, Greece

Background: The measurement of ADAMTS13(A Disintegrin and Metalloprotease with ThromboSpondin type-1 repeats)activity constitutes a cornerstone in the differential diagnosis and monitoring of thrombotic microangiopathies(TMA).

Aims: This study evaluates the utility of ADAMTS13 activity in the routine clinical management of patients during pregnancy and childbirth.

Methods: Patients referred for ADAMTS13 measurement over the last seven years were studied.ADAMTS13 activity and inhibitors were measured using an ELISA method(Technozym,Diapharma).Patients' clinical data during pregnancy were retrospectively collected from treating physicians,who determined the therapeutic management.Thrombotic Thrombocytopenic Purpura(TTP) was defined as TMA with severe ADAMTS13 deficiency(activity≤10%).

Results: Out of 1045 ADAMTS13 activity measurements,28 were performed on 11 patients during pregnancy and childbirth.In 5 patients,TMA manifested for the first-time during pregnancy(4 in the third trimester,1 in the second)with ADAMTS13 < 20%.They were successfully treated with plasma exchange and corticosteroids(addition of rituximab in 1 of 5,with high inhibitor levels),resulting in complete resolution of the syndrome and successful delivery.During follow-up,1 patient experienced a syndrome recurrence 16 months postpartum,which was successfully treated with plasma exchange,rituximab and caplacizumab.In 6 patients,ADAMTS13 activity was measured during pregnancy due to a previous episode,along with regular laboratory monitoring.Among them,4 had a normal delivery,1 had a cesarean section and all delivered full-term infants of normal weight.1 patient delivered prematurely at 29 weeks due to obstetric issues,unrelated to TTP.Another patient,with a history of HELLP syndrome resulting in the termination of a previous pregnancy and successful treatment with 4 sessions of plasma exchange during childbirth,developed TMA at the 18th week of pregnancy with low ADAMTS13(10%).Due to strict measures during the COVID-19 pandemic,the patient was treated only with plasma infusions and corticosteroids,leading to an increase in ADAMTS13(>30%)and a successful delivery by cesarean section.

Conclusion(s): The measurement of ADAMTS13 activity is crucial for therapeutic decisions.From the Greek experience,the possibility of successful treatment with rituximab assistance,when necessary,is evident.The role of new agents,such as caplacizumab,remains to be explored.