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2017 ; 44
(2
): 70-76
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Thrombosis in Inherited Fibrinogen Disorders
#MMPMID28503122
Korte W
; Poon MC
; Iorio A
; Makris M
Transfus Med Hemother
2017[Apr]; 44
(2
): 70-76
PMID28503122
show ga
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.