Jjml. Hoffmann et al., HEREDITARY INCREASE OF PLASMA HISTIDINE-RICH GLYCOPROTEIN ASSOCIATED WITH ABNORMAL HEPARIN-BINDING (HRG EINDHOVEN), Thrombosis and haemostasis, 70(6), 1993, pp. 894-899
Plasma histidine-rich glycoprotein (HRG) was found to be persistently
increased in a patient with a history of recurrent arterial thromboemb
olic events. The mean concentration was 270% of normal pooled plasma.
Increased HRG was found in eight of the 17 relatives studied, but none
of them has experienced thrombo-embolism yet. Apparently, increased H
RG was hereditary with autosomal dominant inheritance. A significant c
orrelation was found between the increased plasma concentration of the
protein and the age of the subjects (P <0.02), whereas no such relati
on is present in a normal population. The plasma HRG of the proposita
and 9 of her family members displayed abnormal binding to heparin, as
assessed in a crossed affinity immuno-electrophoresis system: the usua
l increase in mobility after binding to heparin was absent. The bindin
g of this variant HRG to plasminogen was normal. This case represents
the first abnormal HRG variant reported and it is proposed to designat
e it: HRG Eindhoven.