Case report: the lupus anticoagulant-hypoprothrombinemia syndrome.
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.
Peacock
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Cancer Therapy and Research Center, San Antonio, Texas 78229.
Levine
S P
SP
D000328:Adult; D000991:Antithrombins / Q000378:metabolism; D001779:Blood Coagulation Factors / Q000032:analysis; D006471:Gastrointestinal Hemorrhage / Q000097:blood* / Q000628:therapy; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000032:analysis*; D008297:Male; D010314:Partial Thromboplastin Time; D011241:Prednisone / Q000627:therapeutic use; D011516:Prothrombin / Q000032:analysis* / Q000378:metabolism; D011517:Prothrombin Time; D013577:Syndrome
D000991:Antithrombins; D001779:Blood Coagulation Factors; D016682:Lupus Coagulation Inhibitor; D011516:Prothrombin; D011241:Prednisone