Lupus Anticoagulant-hypoprothrombinemia Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus: A Case Report
- Author:
Yeonah SUL
1
;
Joo Hoon LEE
;
Young Seo PARK
;
Kyung Nam KOH
;
Ho Joon IM
;
Jong Jin SEO
Author Information
1. Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea. yspark@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Lupus anticoagulant;
Hypoprothrombinemia;
Systemic lupus erythematosus
- MeSH:
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
- From:Clinical Pediatric Hematology-Oncology
2014;21(2):145-148
- CountryRepublic of Korea
- Language:Korean
-
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.
