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Thromb Haemost 1993; 70(02): 263-265
DOI: 10.1055/s-0038-1649562
Original Articles
Clinical Studies
Schattauer GmbH Stuttgart

Congenital Histidine-Rich Glycoprotein Deficiency

Toshio Shigekiyo
1   The First Department of Internal Medicine, School of Medicine, the University of Tokushima, Tokushima, Japan
,
Tsutomu Ohshima
2   The Department of Neurosurgery, Anan Kyoei Hospital, Tokushima, Japan
,
Hirofumi Oka
2   The Department of Neurosurgery, Anan Kyoei Hospital, Tokushima, Japan
,
Akira Tomonari
1   The First Department of Internal Medicine, School of Medicine, the University of Tokushima, Tokushima, Japan
,
Hiroyuki Azuma
1   The First Department of Internal Medicine, School of Medicine, the University of Tokushima, Tokushima, Japan
,
Shiro Saito
1   The First Department of Internal Medicine, School of Medicine, the University of Tokushima, Tokushima, Japan
› Author Affiliations
Further Information

Publication History

Received 12 November 1992

Accepted after revision 16 February 1993

Publication Date:
04 July 2018 (online)

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Summary

The proband, a 43-year-old woman, suffered from right transverse sinus thrombosis during oral contraceptive treatment. A month after stopping the drug, her plasma activities of antithrombin III, protein C, protein S, heparin cofactor II, plasminogen and plasminogen activator inhibitor were normal, but her plasma histidine-rich glycoprotein (HRG) level was only 21% of the normal level of 109.5 ± 51.5% (mean ± 2 SD). The HRG concentrations in her plasma determined on four different occasions over 6 months were similar. She showed no clinical signs of liver insufficiency or sepsis. Low levels of plasma HRG (20% to 35% of normal) were also found in her aunt, uncle and two daughters. These results suggest that congenital HRG deficiency is inheritary in this family.

 

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