Download PDF
Thromb Haemost 1976; 36(03): 537-541
DOI: 10.1055/s-0038-1648074
Original Article
Schattauer GmbH

Bleeding Disorder with Abnormal Wound Healing, Acid-Soluble Clots and Normal Factor XIII

Stefan Ragaz
1   Central Haematology Laboratory, Inselspital, CH-3010 Bern, Switzerland
2   Department of Biochemistry, University, St. Andrews, Fife, KYI6 9AL, Scotland
,
Graham Kemp
1   Central Haematology Laboratory, Inselspital, CH-3010 Bern, Switzerland
2   Department of Biochemistry, University, St. Andrews, Fife, KYI6 9AL, Scotland
,
Miha Furlan
1   Central Haematology Laboratory, Inselspital, CH-3010 Bern, Switzerland
2   Department of Biochemistry, University, St. Andrews, Fife, KYI6 9AL, Scotland
,
Eugene A. Beck
1   Central Haematology Laboratory, Inselspital, CH-3010 Bern, Switzerland
2   Department of Biochemistry, University, St. Andrews, Fife, KYI6 9AL, Scotland
› Author Affiliations
Further Information

Publication History

Received 08 March 1976

Accepted 06 July 1976

Publication Date:
03 July 2018 (online)

    Permissions and Reprints

Summary

An unusual bleeding disorder clinically resembling factor XIII deficiency is presented. The only detectable coagulation abnormality was rapid clot dissolution in 1% monochloroacetic acid. This abnormality was ascribed to the sustained increase of a pepsin-like plasma protease which is activated at low pH. A systematic search for similar phenomena revealed that massive blood transfusion may also enhance plasma-clot solubility in acid, possibly by release of a red cell protease. We conclude that the acid clot solubility test is not a specific indicator of factor XIII deficiency, but this simple assay is recommended for further studies of acid plasma protease activity. The diagnostic relevance and pathophysiologic importance of increased pepsin-like activity in plasma remain to be elucidated.

 

  • References

  • 1 Anson M. L. 1938; The estimation of pepsin, trypsin, papain, and cathepsin with hemoglobin. Journal of General Physiology 22: 79.
    CrossrefPubMedGoogle Scholar
  • 2 Clauss A. 1957; Gerinnungsphysiologische Schnellmethode zur Bestimmung des Fibrinogens. Acta Haematologica 17: 237.
    CrossrefPubMedGoogle Scholar
  • 3 Ikemori R, Gruhl M, Shrivastava S, Shanberge J. N. 1975; Solubility of fibrin clots in monochloracetic acid. A reflection of serum pepsinogen levels. American Journal of Clinical Pathology 63: 49.
    CrossrefPubMedGoogle Scholar
  • 4 Hirschowitz B. I, Arbor A. 1955; Pepsinogen in the blood. Journal of Laboratory and Clinical Medicine 46: 568.
    PubMedGoogle Scholar
  • 5 Laki K, Lorand L. 1948; On the solubility of fibrin clots. Science 108: 280.
    CrossrefPubMedGoogle Scholar
  • 6 Lorand L, Urayama T, De Kiewiet J. W. C, Nossel H. L. 1969; Diagnostic and genetic studies on fibrin-stabilizing factor with a new assay based on amine incorporation. Journal of Clinical Investigation 48: 1054.
    CrossrefPubMedGoogle Scholar
  • 7 McKee P. A, Mattock P, Hill R. L. 1970; Subunit structure of human fibrinogen, soluble fibrin, and crosslinked insoluble fibrin. Proceedings of the National Academy of Sciences (Washington) 66: 738.
    CrossrefPubMedGoogle Scholar
  • 8 Reichelt D, Jacobsohn E, Haschen R. J. 1974; Purification and properties of cathepsin D from human erythrocytes. Biochimica et Biophysica Acta 341: 15.
    CrossrefPubMedGoogle Scholar
  • 9 Shanberge J. N, Ikemori R, Shrivastava S, Gruhl M. C. 1973; Further studies on the solubility of fibrin clots in monochloracetic acid. Federation Proceedings 32: 290.
    PubMedGoogle Scholar
  • 10 Tyler H. M. 1966; A comparative study of the solvents commonly used to detect fibrin stabilization. Thrombosis et Diathesis haemorrhagica (Stuttg.) 16: 61.
    PubMedGoogle Scholar