Lupus anticoagulant-hypoprothrombinemia syndrome: report of two cases and review of the literature.
Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare acquired disorder caused by prothrombin antibodies. The disease is most common in the pediatric age group (<16 years), and more prevalent in women. There are well-established clinical diseases associated with LA-HPS, most notably systemic lupus erythematosus (SLE) and viral infections. The clinical manifestation of LA-HPS varies greatly in severity and it may cause severe life-threatening bleeding diathesis. LA-HPS is to be suspected when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant. The diagnosis is confirmed in the laboratory by identification of reduced prothrombin levels. There are no standardized recommendations for treatment of the hemorrhage associated with the syndrome; corticosteroids are used as first-line treatment. This review summarizes what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of LA-HPS, and presents two case reports.
Coagulation Laboratory, Department of Clinical Chemistry, Microbiology and Immunology, Ghent University Hospital, Ghent, Belgium.
S M N
SM
Mulliez
Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
F
F
De Keyser
Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
C
C
Verbist
Department of Hematology, Ghent University Hospital, Ghent, Belgium.
A
A
Vantilborgh
Department of Hematobiology, Erasme Hospital, Brussels, Belgium.
W
W
Wijns
Department of Hematobiology, Erasme Hospital, Brussels, Belgium.
I
I
Beukinga
Coagulation Laboratory, Department of Clinical Chemistry, Microbiology and Immunology, Ghent University Hospital, Ghent, Belgium katrien.devreese@uzgent.be.
K M J
KM
Devreese
D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000097:blood*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007020:Hypoprothrombinemias / Q000097:blood*; D016682:Lupus Coagulation Inhibitor / Q000097:blood*; D008180:Lupus Erythematosus, Systemic / Q000097:blood*; D008297:Male
D017152:Antibodies, Antiphospholipid; D016682:Lupus Coagulation Inhibitor
Lupus anticoagulant; acquired hypoprothrombinemia; antiprothrombin antibodies; bleeding; hypoprothrombinemia; prothrombin