Clin Pediatr Hematol Oncol.  2014 Oct;21(2):145-148. 10.0000/cpho.2014.21.2.145.

Lupus Anticoagulant-hypoprothrombinemia Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus: A Case Report

• Sul Y
• Lee JH
• Park YS
• Koh KN
• Im HJ
• Seo JJ

• KMID: 2279825
• DOI: http://doi.org/10.0000/cpho.2014.21.2.145

Abstract

The lupus anticoagulant-hypoprothrombinemia syndrome, characterized by presence of lupus anticoagulant with acquired factor II deficiency, is a rare disease entity often presented with acute bleeding episodes. A 15-year-old girl was hospitalized with 3 month history of menorrhagia and easy bruising. Prothrombin time (31.3 sec, normal value: 10-13 sec) and activated partial thromboplastin time (72.5 sec, normal value: 27.5-34.7 sec) were markedly prolonged and partially corrected after mixing with normal plasma. Decreased Factor II activity (4%, normal range: 79-131%) or prolonged dilute Russell's viper venom time (89.8 sec, normal value: 25.4-34.3 sec), was consistent with lupus anticoagulant-hypoprothrombinemia syndrome. Antinuclear antibody, anti-double strand-DNA antibodies and anticardiolipin antibodies were also positive. Bleeding diathesis tends to wax and wane while 5 years of treatment with steroid combined with immunosuppressants, however, there was no more active bleeding episodes. Several years after diagnosis, myocarditis, pericarditis, seizure was occurred, fulfilled the diagnostic criteria of systemic lupus erythematosus.

Keyword

Lupus anticoagulant; Hypoprothrombinemia; Systemic lupus erythematosus

MeSH Terms

Adolescent
Antibodies
Antibodies, Anticardiolipin
Antibodies, Antinuclear
Diagnosis
Disease Susceptibility
Female
Hemorrhage
Humans
Hypoprothrombinemias
Immunosuppressive Agents
Lupus Coagulation Inhibitor
Lupus Erythematosus, Systemic*
Menorrhagia
Myocarditis
Partial Thromboplastin Time
Pericarditis
Plasma
Prothrombin
Prothrombin Time
Rare Diseases
Reference Values
Seizures
Antibodies
Antibodies, Anticardiolipin
Antibodies, Antinuclear
Immunosuppressive Agents
Lupus Coagulation Inhibitor
Prothrombin