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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)

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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.

 

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