Bleeding disorder with abnormal wound healing, acid-soluble clots and normal factor XIII.
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. Asystematic 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.
D000085:Acetates; D000328:Adult; D001778:Blood Coagulation Disorders / Q000097:blood*; D005176:Factor XIII / Q000032:analysis*; D005177:Factor XIII Deficiency; D005260:Female; D005337:Fibrin; D006801:Humans; D010447:Peptide Hydrolases / Q000097:blood; D012995:Solubility; D014945:Wound Healing
D000085:Acetates; D005337:Fibrin; D005176:Factor XIII; D010447:Peptide Hydrolases