Thromboembolism complicating the treatment of lupus anticoagulant hypoprothrombinemia syndrome.
Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare disorder characterized by a bleeding tendency due to factor II deficiency associated with the presence of lupus anticoagulant (LAC) autoantibodies. We describe a patient with systemic lupus erythematosus and LAHPS in whom successful treatment of central nervous system bleeding due to severe factor II deficiency was followed by a major thromboembolic complication. Literature review revealed 2 other patients with LAHPS who developed thrombosis resulting from the treatment of factor II deficiency. We suggest that factor II deficiency counterbalances the prothrombotic effect of LAC in LAHPS, and correcting this deficiency may promote thromboembolism.
Vinet
Evelyne
E
Division of Rheumatology, Centre Hospitalier de l'Université de Montréal, Department of Medicine, University of Montreal School of Medicine, Montreal, Quebec, Canada.
Rich
Eric
E
Senécal
Jean-Luc
JL
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000175:diagnosis / Q000378:metabolism*; D001379:Azathioprine / Q000627:therapeutic use; D002493:Central Nervous System Diseases / Q000209:etiology / Q000628:therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy; D006801:Humans; D007020:Hypoprothrombinemias / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000378:metabolism; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016682:Lupus Coagulation Inhibitor / Q000378:metabolism*; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000378:metabolism*; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D013577:Syndrome; D013923:Thromboembolism / Q000209:etiology*
D000305:Adrenal Cortex Hormones; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016682:Lupus Coagulation Inhibitor; D003520:Cyclophosphamide; D001379:Azathioprine; D011241:Prednisone