Subscribe to RSS
DOI: 10.1055/s-0038-1649562
Congenital Histidine-Rich Glycoprotein Deficiency
Publication History
Received 12 November 1992
Accepted after revision 16 February 1993
Publication Date:
04 July 2018 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00035024/199302/lookinside/thumbnails/10-1055-s-0038-1649562-1.jpg)
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.
-
References
- 1 Heimburger N, Haupt H, Kranz T, Baudner S. Human serum proteins with a high affinity for carboxymethylcellulose, II: Physicochemical and immunological characterization of a histidine-rich 3.8 S α2 glycoprotein (CM protein I). Hoppe-Seyler’s Z Physiol Chem 1972; 353: 1133-1140
- 2 Koide T, Foster D, Yoshitake S, Davie EW. Amino acid sequence of human histidine-rich glycoprotein derived from the nucleotide sequence of its cDNA. Biochemistry 1986; 25: 2220-2225
- 3 Koide T. Human histidine-rich glycoprotein gene: Evidence for evolutionary relatedness to cystatin supergene family. Thromb Res 1988; (Suppl. 08) 91-97
- 4 Morgan WT. Human serum histidine-rich glycoprotein. I. Interactions with heme, metal ions and organic ligands. Biochim Biophys Acta 1978; 535: 319-333
- 5 Lijnen HR, Hoylaerts M, Collen D. Isolation and characterization of a human plasma protein with affinity for the lysine binding sites in plasminogen. Role in the regulation of fibrinolysis and identification as histidine-rich glycoprotein. J Biol Chem 1980; 255: 10214-10222
- 6 Leung LLK, Nachman RL, Harpel PC. Complex formation of platelet thrombospondin with histidine-rich glycoprotein. J Clin Invest 1984; 73: 5-12
- 7 Leung LLK. Interaction of histidine-rich glycoprotein with fibrinogen and fibrin. J Clin Invest 1986; 77: 1305-1311
- 8 Lerch PG, Nydegger UE, Kuyas C, Haeberli A. Histidine-rich glycoprotein binding to activated human platelets. Br J Haematol 1988; 70: 219-224
- 9 Saigo K, Shatsky M, Levitt LJ, Leung LLK. Interaction of histidine-rich glycoprotein with human T lymphocytes. J Biol Chem 1989; 264: 8249-8253
- 10 Engesser L, Kluft C, Briét E, Brommer EJP. Familial elevation of plasma histidine-rich glycoprotein in a family with thrombophilia. Br J Haematol 1987; 67: 355-358
- 11 Schleider MA, Nachman RL, Jaffe EA, Coleman M. A clinical study of the lupus anticoagulant. Blood 1976; 48: 499-509
- 12 Exner T, Rickard KA, Kronenberg H. A sensitive test demonstrating lupus anticoagulant and its behavioural patterns. Br J Haematol 1978; 40: 143-151
- 13 Laurell CB. Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies. Anal Biochem 1966; 15: 45-52
- 14 Lijnen HR, Jacobs G, Collen D. Histidine-rich glycoprotein in a normal and a clinical population. Thromb Res 1981; 22: 519-523
- 15 Silverstein RL, Nachman RL, Leung LLK, Harpel PC. Activation of immobilized plasminogen by tissue activator. Multimolecular complex formation. J Biol Chem 1985; 260: 10346-10352
- 16 Vestergaard AB, Andersen HF, Magnusson S, Halkier T. Histidine-rich glycoprotein inhibits contact activation of blood coagulation. Thromb Res 1990; 60: 385-396
- 17 Kazama Y, Koide T. Modulation of protein C inhibitor activity by histidine-rich glycoprotein and platelet factor 4: Role of zinc and calcium ions in the heparin-neutralizing ability of histidine-rich glycoprotein. Thromb Haemostas 1992; 67: 50-55
- 18 Thaler E, Lechner K. Antithrombin III deficiency and thromboembolism. Clin Hematol 1981; 10: 369-390
- 19 Bovill EG, Bauer KA, Dickerman JD, Callas P, West B. The clinical spectrum of heterozygous protein C deficiency in a large new England kindred. Blood 1989; 73: 712-717
- 20 Engesser L, Broekmans AW, Briët E, Brommer EJP, Bertina RM. Hereditary protein S deficiency: clinical manifestations. Ann Intern Med 1987; 106: 677-682
- 21 Girolami A, Simioni P, Girolami B, Zanardi S. The role of drugs, particularly oral contraceptives, in triggering thrombosis in congenital defects of coagulation inhibitors: a study of six patients. Blood Coag Fibrinol 1991; 2: 673-678
- 22 Jespersen J, Kluft C. Decreased levels of histidine-rich glycoprotein (HRG) and increased levels of free plasminogen in women on oral contraceptives low in estrogen. Thromb Haemostas 1982; 48: 283-285