TMH452864_sm_Suppl._Material.pdf (644.41 kB)
Supplementary Material for: Thrombosis in Inherited Fibrinogen Disorders
dataset
posted on 2017-03-09, 12:37 authored by Korte W., Poon M.-C., Iorio A., Makris M.Although inherited fibrinogen disorders (IFD) are primarily
considered to be bleeding disorders, they are associated
with a higher thrombotic complication risk than defects
in other clotting factors. Managing IFD patients
with thrombosis is challenging as anticoagulant treatment
may exacerbate the underlying bleeding risk which
can be life-threatening. Due to the low prevalence of IFD,
there is little information on pathophysiology or optimal
treatment of thrombosis in these patients. We searched
the literature for cases of thrombosis among IFD patients
and identified a total of 128 patient reports. In approximately
half of the cases, thromboses were spontaneous,
while in the others trauma, surgery, and parturition contributed
to the risk. The true mechanism(s) of thrombosis
in IFD patients remain to be elucidated. A variety of
anticoagulant treatments have been used in the treatment
or prevention of thrombosis, sometimes with concurrent
fibrinogen replacement therapy. There is no definite
evidence that fibrinogen supplementation increases
the risk of thrombosis, and it may potentially be effective
in the treatment and prevention of both thrombosis and
hemorrhage in IFD patients.