Case report: the lupus anticoagulant-hypoprothrombinemia syndrome.

Abstract

The lupus anticoagulant is a well-described in vitro phenomenon that may be associated with arterial and venous thrombotic episodes. The lupus anticoagulant is never accompanied by a hemorrhagic diathesis unless it is associated with a second coagulation abnormality such as thrombocytopenia or hypoprothrombinemia. The lupus anticoagulant-hypoprothrombinemia syndrome is now a well-defined entity that may cause a severe, life-threatening hemorrhagic diathesis. The hypoprothrombinemia in this syndrome is the result of rapid clearance of prothrombin-antiprothrombin antibody complexes by the reticulo-endothelial system. The cause of antiprothrombin antibody formation is unknown. The authors describe a recent experience with a patient with this syndrome who initially had recurrent, life-threatening gastrointestinal bleeding. They were able to demonstrate hypoprothrombinemia and the presence of prothrombin-antiprothrombin antibody immune complexes. The patient was treated with prednisone, with correction of the bleeding disorder; however, the patient had resultant death from thrombosis. A literature review of the past 30 years as it relates to the discovery and treatment of this phenomenon is included.

• Authors: N W Peacock, S P Levine
• Journal: The American journal of the medical sciences (Am J Med Sci) Vol. 307 Issue 5 Pg. 346-9 (May 1994) ISSN: 0002-9629 [Print] United States
• PMID: 8172228 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Antithrombins
• Blood Coagulation Factors
• Lupus Coagulation Inhibitor
• Prothrombin
• Prednisone

Topics

• Adult
• Antithrombins (metabolism)
• Blood Coagulation Factors (analysis)
• Gastrointestinal Hemorrhage (blood, therapy)
• Humans
• Lupus Coagulation Inhibitor (analysis)
• Male
• Partial Thromboplastin Time
• Prednisone (therapeutic use)
• Prothrombin (analysis, metabolism)
• Prothrombin Time
• Syndrome