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Structural characterization and functional effects of a circulating heparan sulfate in a patient with hepatocellular carcinoma.
- Source :
-
American journal of hematology [Am J Hematol] 1998 Aug; Vol. 58 (4), pp. 285-92. - Publication Year :
- 1998
-
Abstract
- A circulating anticoagulant was isolated from the plasma of a 42-year-old man with cirrhosis and hepatocellular carcinoma who had an unusual coagulation test profile. The patient developed a fatal coagulopathy, unresponsive to protamine therapy or plasma exchange following liver biopsy. However, at presentation, routine hemostasis assays were normal. The patient had mucocutaneous bleeding but the sole laboratory abnormality was a prolonged thrombin time (TT = 99 s, normal 25-35 s). Protamine titration indicated activity equivalent to a heparin concentration of 6-7 U/ml. Antithrombin III (AT III) antigen and activity were markedly elevated. The anticoagulant activity, purified from plasma by DEAE chromatography, was identified as a glycosaminoglycan (GAG). GAG anti-thrombin activity was completely abolished by heparin lyase III. Based on the degree of sulfation and HPLC pattern, the GAG was classified as heparan sulfate. Low levels (4 microM) of purified GAG markedly prolonged the TT (>120 s) but not the activated partial thromboplastin time (PTT) (31.4 s). In a Factor Xa assay, the GAG exhibited a potency equivalent to 0.06 U of low molecular weight heparin per nmol of uronic acid. Patients with endogenous circulating glycosaminoglycans can present with unusual laboratory coagulation test profiles. These reflect complex dysfunction of hemostasis, leading to difficulty in providing diagnosis and effective care.
- Subjects :
- Adult
Blood Coagulation drug effects
Blood Platelets drug effects
Blood Platelets physiology
Disaccharides analysis
Factor Xa drug effects
Glycosaminoglycans blood
Glycosaminoglycans chemistry
Glycosaminoglycans pharmacology
Heparitin Sulfate chemistry
Humans
Male
Partial Thromboplastin Time
Protein Conformation
Receptors, Thrombin drug effects
Sulfuric Acid Esters analysis
Thrombin drug effects
Thrombin Time
Carcinoma, Hepatocellular blood
Heparitin Sulfate blood
Subjects
Details
- Language :
- English
- ISSN :
- 0361-8609
- Volume :
- 58
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- American journal of hematology
- Publication Type :
- Academic Journal
- Accession number :
- 9692391
- Full Text :
- https://doi.org/10.1002/(sici)1096-8652(199808)58:4<285::aid-ajh6>3.0.co;2-m