ISTH Congress Abstracts

Official abstracts site for the ISTH Congress

MENU 
  • Home
  • Congress Archive
    • ISTH 2021 Congress
    • ISTH 2020 Congress
  • Resources
  • Search

von Willebrand Disease Combined with Other Coagulation Defects: Experience at a Single Reference Coagulation Laboratory in Iran

O. Seidi Zadeh, M. Ahmadinejad, S. Homayoun, M. Mojtabavi, S. Mohsenian

Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran, Islamic Republic of

Abstract Number: PB1546

Meeting: ISTH 2020 Congress

Theme: Platelet Disorders and von Willebrand Disease » VWF and von Willebrand Factor Disorders - Clinical Conditions

Background: Von Willebrand disease (VWD) is known as the most common inherited bleeding disorder however, well-documented cases of combined VWD with other coagulation defects have been infrequently reported.

Aims: To describe a series of patients with combined VWD and coagulation defects and also evaluate the incidence of combined factor deficiencies in Iran.

Methods: All subjects with VWD panel test requests from June 2018 – May 2019 were included in this prospective monocentric study. For all subjects, demographic data, PT, PTT, VWF: Ag, VWF: RCo, FVIII: C were evaluated by standard methods. Patients with prolonged PT and/or PTT were further investigated for other factor assays and lupus anticoagulant (LA) panel. Also ISTH- BAT, RIPA, and VWF: CB for patients with confirmed VWD results were performed. The patients receiving FVIII or VWF concentrate in 10 days before sampling and pregnant women were excluded.

Results: 1107 subjects were evaluated which results of 70 cases showing VWF: Ag or/and VWF: RCo ≤ 40% and VWD diagnosis was confirmed. After further investigation, we identified seven patients with a combination of coagulation defects and VWD (Table1). All patients except one had significant clinical symptoms (median: 10 and range: 4-21) with at least three hemorrhagic symptoms that might be a result of combined deficiencies. Interestingly in two patients LA was positive. This study providing an estimated incidence of 8.5 % for combined deficiencies in our patient population with the almost same prevalence in men and women.

Conclusions: We can conclude that combined factor deficiencies incidence in our patient population is higher than other records due to consanguineous marriages and it seems to be underdiagnosed. As Co-inheritance of VWD and factor deficiencies can alter the treatment approach for the management of patients, it worth attention.

Case Clinical presentation FVIII:C (IU/mL) VWF:Ag (IU/mL) VWF:RCo (IU/mL) VWF:CB (IU/mL) BS coagulation defect(s)
1/5 year/M Postoperative bleeding 54 34 29 36 21 LA: Positive
2/4 year/F Epistaxis, Oral cavity, GI bleeding 64 61 35 36 20 FVII: 34, LA: Positive
3/5 year/M Postoperative bleeding 78 42 34 28 10 FVII: 42
4 /27 year/F Epistaxis, Menorrhagia 75 43 35 55 7 FXI: 2
5/31 year/F Mucocutaneous bleeding, Tooth extraction 43 50 36 53 7 FVII: 11
6/32 year/M Mucocutaneous bleeding, Tooth extraction 70 45 32 41 4 FVII: 52
7/5 year/M Epistaxis, Mucocutaneous bleeding 74 40 30 39 2 FXII:42

[Table 1. Clinical presentation and von Willebrand disease screening in patients with combined coagulation defects.]

To cite this abstract in AMA style:

Seidi Zadeh O, Ahmadinejad M, Homayoun S, Mojtabavi M, Mohsenian S. von Willebrand Disease Combined with Other Coagulation Defects: Experience at a Single Reference Coagulation Laboratory in Iran [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/von-willebrand-disease-combined-with-other-coagulation-defects-experience-at-a-single-reference-coagulation-laboratory-in-iran/. Accessed May 16, 2022.

« Back to ISTH 2020 Congress

ISTH Congress Abstracts - https://abstracts.isth.org/abstract/von-willebrand-disease-combined-with-other-coagulation-defects-experience-at-a-single-reference-coagulation-laboratory-in-iran/

Simple Search

Supported By:

Bristol Myers Squibb and Pfizer

ISTH 2021 Congress site

Visit the official web site for the ISTH 2021 Virtual Congress »

  • Help & Support
  • About Us
  • Cookies & Privacy
  • Wiley Job Network
  • Terms & Conditions
  • Advertisers & Agents
Copyright © 2022 John Wiley & Sons, Inc. All Rights Reserved.
Wiley