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Severe thrombotic tendency associated with a type I plasminogen deficiency
F W Leebeek1, E A Knot, J W Ten Cate
1Department of Internal Medicine II, University Hospital Dijkzigt, Rotterdam, The Netherlands.
American Journal of Hematology
|January 1, 1989
Summary
A patient with recurrent deep vein thrombosis had isolated hypoplasminogenemia, a deficiency in plasminogen synthesis. This condition, despite a normal plasminogen molecule, indicates a severe thrombotic tendency.
Area of Science:
- Hematology
- Thrombosis Research
- Molecular Biology
Background:
- Recurrent spontaneous deep vein thrombosis (DVT) poses a significant clinical challenge.
- Understanding the underlying hemostatic abnormalities is crucial for effective management.
Observation:
- A 45-year-old female presented with recurrent spontaneous DVT.
- Laboratory analysis revealed isolated hypoplasminogenemia, with plasminogen activity at 42% and antigen levels at 37%.
Findings:
- The patient's plasminogen molecule was confirmed as normal in function and structure.
- All other tested hemostatic parameters were within normal limits.
- Desmopressin acetate (DDAVP) stimulation showed normal responses for tissue plasminogen activator and factor VIIIR:WF.
Implications:
- The hypoplasminogenemia appears to result from decreased synthesis of a normal plasminogen molecule.
- This isolated deficiency is strongly associated with a severe thrombotic tendency.
- Further research into plasminogen synthesis defects may reveal novel therapeutic targets for thrombosis.