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Poster Networking

Modulators of Coagulation and von Willebrand Factor

PB0360 - Acquired von Willebrand syndrome in a patient with monoclonal gammopathy of undetermined significance.

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

    Co-Author(s)

  • CM

    Ciro Miele

    Biologist
    Department of Integrated Activity of Laboratory Medicine and Transfusion, University of Naples "Federico II", Naples, Italy.
    Naples, Campania, Italy

    • Co-Author(s)

  • PC

    Paolo Conca

    assistant professor
    DEPARTMENT OF CLINICAL MEDICINE AND SURGERY. Federico II University of Naples
    Naples, Campania, Italy

  • FC

    Filomena Capasso

    biologist
    2. UOC Laboratory medicine of hematology and hemostasis; Federico II University Hospital, Naples, Italy
    Naples, Campania, Italy

  • CM

    Cristina Mazzacara

    biologist
    Department of Integrated Activity of Laboratory Medicine and Transfusion, University of Naples "Federico II", Naples, Italy.
    Naples, Campania, Italy

  • RM

    Rosaria Mormile

    Biologist
    (1) Hematology, Department of Translational and precision Medicine, Sapienza University, Rome, Italy
    Rome, Lazio, Italy

  • FD

    Francesca D'Auria

    biologist
    4. Department of Molecular Medicine and Medical Biotechnology, University of Naples “Federico II”, Naples, Italy
    Naples, Campania, Italy

  • LM

    Luca Manfredi

    biologist
    Department of Integrated Activity of Laboratory Medicine and Transfusion, University of Naples "Federico II", Naples, Italy.
    Naples, Campania, Italy

  • GM

    Gaetano Mastranzo

    biologist
    Department of Molecular Medicine and Medical Biotechnology, University of Naples “Federico II”, Naples, Italy
    Naples, Campania, Italy

  • LV

    Luigi junior Valletta

    RESIDENT
    DEPARTMENT OF CLINICAL MEDICINE AND SURGERY. Federico II University of Naples
    Naples, Campania, Italy

  • RR

    Raffaele Russo

    RESIDENT
    DEPARTMENT OF CLINICAL MEDICINE AND SURGERY. Federico II University of Naples
    Naples, Campania, Italy

  • GC

    Greta Chiarelli

    RESIDENT
    DEPARTMENT OF CLINICAL MEDICINE AND SURGERY. Federico II University of Naples
    Naples, Campania, Italy

  • EC

    Ernesto Cimino

    assistant professor
    DEPARTMENT OF CLINICAL MEDICINE AND SURGERY. Federico II University of Naples
    Naples, Campania, Italy

  • MD

    Matteo Di Minno

    FULL PROFESSOR
    DEPARTMENT OF CLINICAL MEDICINE AND SURGERY. Federico II University of Naples
    Naples, Campania, Italy

    • Presenting Author(s)

  • Antonella Tufano, MD, PhD

    ASSISTANT PROFESSOR
    DEPARTMENT OF CLINICAL MEDICINE AND SURGERY , FEDERICO II UNIVERSITY OF NAPLES
    Naples, Campania, Italy

Background: Acquired von Willebrand syndrome (AVWS) is a rare, acquired bleeding disorder resembling inherited von Willebrand disease (VWD), that occurs in patients with no personal/family history of bleeding. In clinical practice it may be very difficult to distinguish between VWD and AVWS, since the bleeding symptoms and laboratory characteristics are similar. However, it is of great importance to make a correct diagnosis as the treatment is different.

Aims: We report a case of a 72-year-old woman affected by AVWS secondary to monoclonal gammopathy of undetermined significance (MGUS), referred to our hemophilia center for post-operative hemorrhagic events.

Methods: A 72-year-old woman suffered from a post-operative hemorrhage following an elective cholecystectomy in May 2023. On re-examination, the medical history revealed a history of menorrhagia, and an episode of hemothorax after tracheal intubation following a hysterectomy in July 2022. There was no family history of bleeding diseases. Laboratory evaluation showed a prolonged aPTT, normal PT, FVIII:C 17%, vWF:Ag 15.5%, RICof 9.6%, and vWF:CB 11.1%. (Table1). Mixing studies failed to detect neutralizing VWF inhibitors. After the infusion of FVIII/vWF concentrate, the observed half-life was short, no more than 6 hours, confirming the diagnosis of AVWS. When IV IG were administered, a rapid and prolonged rise of FVIII:C, vWF:Ag and RICof was obtained. Subsequent investigation for an underlying disorder revealed a MGUS IgG-λ type.

Results: In this case report pharmacokinetic studies along with mixing studies were diriment for confirming the diagnosis of AVWS

Conclusion(s): AVWS is among hematological disorders secondary to MGUS, and despite being reported also in other lymphoproliferative disorders, MGUS is the condition most frequently associated (one third of cases). Treatment of AVWS is focused on the underlying disease, the treatment of acute episodes and the prevention of bleedings in high-risk situations (i.e. surgery). However, when considering MGUS, the eradication is hard to reach or even treat.