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Case Report

Thromboembolism Complicating the Treatment of Lupus Anticoagulant Hypoprothrombinemia Syndrome

ÉVELYNE VINET, ÉRIC RICH, and JEAN-LUC SENÉCAL

ABSTRACT.

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. (First Release Aug 15 2006; J Rheumatol 33:2088-90)

Key Indexing Terms:

LUPUS ANTICOAGULANT HYPOPROTHROMBINEMIA SYNDROME
THROMBOSIS

FACTOR II DEFICIENCY
ANTIPHOSPHOLIPID SYNDROME

SYSTEMIC LUPUS ERYTHEMATOSUS
AUTOANTIBODIES


From the Division of Rheumatology, Centre Hospitalier de l'Université de Montréal, Department of Medicine, University of Montreal School of Medicine, Montreal, Quebec, Canada.

Supported by a Canadian Institutes of Health Research grant (MOP-62687).

E. Vinet, MD, FRCPC, Senior Rheumatology Fellow; E. Rich, MD, FRCPC, Assistant Professor of Medicine; J-L. Senécal, MD, FRCPC, FACP, Professor of Medicine, University of Montreal School of Medicine, Director, Laboratory for Research in Autoimmunity, Centre Hospitalier de l'Université de Montréal.

Address reprint requests to Dr. J-L. Senécal, Hôpital Notre-Dame (M-4243), Centre Hospitalier de l'Université de Montréal, 1560 Sherbrooke Street East, Montreal, Quebec H2L 4M1, Canada. E-mail: jeanluc.senecal@sympatico.ca

Accepted for publication May 23, 2006.




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