Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019 Nov;187(4):509-517.
doi: 10.1111/bjh.16097. Epub 2019 Jul 14.

Bleeding and bruising in Osteogenesis Imperfecta: International Society on Thrombosis and Haemostasis bleeding assessment tool and haemostasis laboratory assessment in 22 individuals

Koert Gooijer  1 , Jan M M Rondeel  2 , Fleur S van Dijk  1   3 , Arjan G J Harsevoort  1 , Guus J M Janus  1 , Anton A M Franken  1
Affiliations

Bleeding and bruising in Osteogenesis Imperfecta: International Society on Thrombosis and Haemostasis bleeding assessment tool and haemostasis laboratory assessment in 22 individuals

Koert Gooijer et al. Br J Haematol. 2019 Nov.

Abstract

Osteogenesis imperfecta (OI) is characterized by susceptibility to bone fractures. Other symptoms, such as easy bruising and bleeding complications during surgery necessitating transfusions, have also been reported. The aim of the cross-sectional pilot study was to assess the bleeding and bruising tendency in OI patients and to screen for possible underlying haematological disorders. Bleeding tendency was investigated using the International Society on Thrombosis and Haemostasis bleeding assessment tool (ISTH-BAT) in 22 adult OI patients. Laboratory testing was performed to investigate for bleeding disorders or abnormal coagulation. Four patients [OI type 1(n = 3), OI type 4(n = 1)] had a bleeding score (BS) fitting with a bleeding tendency, but without test results pointing to a coagulopathy. Two patients [OI type 1(n = 1), OI type 3 (n = 1)] without a bleeding tendency according to the BS had increased fibrinolysis. This is the second largest study to date addressing bleeding tendency in OI and the first study to use ISTH-BAT and elaborate laboratory testing for coagulopathies. Four patients had an increased bleeding tendency. However, laboratory testing demonstrated no bleeding disorder or abnormal coagulation. Increased fibrinolysis was demonstrated in two patients without bleeding tendency on BS. Vascular fragility as a cause of bleeding tendency in OI has been suggested earlier. Further research on bleeding tendency in OI is important.

Keywords: Osteogenesis Imperfecta; blood coagulation disorders; capillary fragility; fibrinolysis; hemorrhage.

PubMed Disclaimer

References

    1. Akkerman, J.W.N. (2006) Betekenis van de ‘ Platelet Function Analyzer-100 ® ’ in de dagelijkse diagnostiek. Nederlands Tijdschrift voor Hematologie, 3, 133-137.
    1. Alves, G.S.A., Orsi, F.A., Santiago-Bassora, F.D., Quaino, S.K.P., Montalvão, S.A.L., de Paula, E.V. & Annichino-Bizzacchi, J.M. (2016) Laboratory evaluation of patients with undiagnosed bleeding disorders. Blood Coagulation & Fibrinolysis, 27, 500-505.
    1. Castellone, D.D. (2017) Establishing reference intervals in the coagulation laboratory. International Journal of Laboratory Hematology, 39, 121-127.
    1. CLSI. (2008). Defining, establishing, and verifying reference intervals in the clinical laboratory; Approved Guideline-Third Edition. CLSI document EP28-A3c. Clinical and Laboratory Standards Institute, Wayne, PA.
    1. Deforest, M., Grabell, J., Albert, S., Young, J., Tuttle, A., Hopman, W.M. & James, P.D. (2015) Generation and optimization of the self-administered bleeding assessment tool and its validation as a screening test for von Willebrand disease. Haemophilia, 21, e384-e388.

LinkOut - more resources