Lupus anticoagulant-hypoprothrombinemia in healthy adult
0
조회
0
다운로드
3
인용
0
참고문헌
키워드
초록
The presence of lupus anticoagulant is associated with an elevated risk of venous and arterial thrombosis, and recurrent miscarriages as well. For some cases, this disease can present with bleeding as a consequence of lupus anticoagulant‐hypoprothrombinemia (LAHPS). LAHPS is a rare disease and it is reported to be most frequent in young females with/without systemic lupus erythematosus or in healthy children who are suffering with a viral infection. In such cases, steroid therapy is usually effective in normalizing the biological abnormalities and controlling the bleeding problems. A 34-year-old previously healthy man was admitted to our department because of his prolonged coagulation times; these abnormalities were discovered before performing orthopedic surgery. The prothrombin time (PT) was 15.2 sec, and the activated partial thromboplastin time (APTT) was 37.7 sec. A 1:1 dilution of patient plasma with normal plasma nearly corrected the PT, but this failed to correct the APTT. Evaluation of the clotting factors revealed decreased levels of factors II, V, VIII, IX and XI. The presence of LA was demonstrated by the dRVVT test, and the patient was diagnosed with LAHPS. He was successfully treated with corticosteroid before performing the orthopedic surgery.
Abstract
The presence of lupus anticoagulant is associated with an elevated risk of venous and arterial thrombosis, and recurrent miscarriages as well. For some cases, this disease can present with bleeding as a consequence of lupus anticoagulant‐hypoprothrombinemia (LAHPS). LAHPS is a rare disease and it is reported to be most frequent in young females with/without systemic lupus erythematosus or in healthy children who are suffering with a viral infection. In such cases, steroid therapy is usually effective in normalizing the biological abnormalities and controlling the bleeding problems. A 34-year-old previously healthy man was admitted to our department because of his prolonged coagulation times; these abnormalities were discovered before performing orthopedic surgery. The prothrombin time (PT) was 15.2 sec, and the activated partial thromboplastin time (APTT) was 37.7 sec. A 1:1 dilution of patient plasma with normal plasma nearly corrected the PT, but this failed to correct the APTT. Evaluation of the clotting factors revealed decreased levels of factors II, V, VIII, IX and XI. The presence of LA was demonstrated by the dRVVT test, and the patient was diagnosed with LAHPS. He was successfully treated with corticosteroid before performing the orthopedic surgery.
참고문헌 (8)
- Cuadrado MJ, "Hughes (antiphospholipid) syndrome: clinical features", Rheumatic Disease Clinics of North America, 27, pp.507~524 (2001)
- Vivaldi P, "Severe bleeding due to acquired hypoprothrombinemia-lupus anticoagulant syndrome: case report and review of literature", Haematologica, 82, pp.345~347 (1997)
- Bernini JC, "Hypoprothrombinemia and severe hemorrhage associated with a lupus anticoagulant", JOURNAL OF PEDIATRICS, 123, pp.937~939 (1993)
- Hift RJ, "Acquired hypoprothrombinaemia and lupus anticoagulant: response to steroid therapy", British Journal of Rheumatology, 30, pp.308~310 (1991)
- Follea G, "Antiprothrombinase and factor II deficiency in a non SLE patient", Thrombosis and Haemostasis, 46, pp.670~ (1981)
- Bajaj SP, "A mechanism for the hypoprothrombinemia of the acquired hypothrombinemia-lupus anticoagulant syndrome", Blood, 61, pp.684~692 (1983)
- Simel DL, "Correction of hypoprothrombinemia by immunosuppressive treatment of the lupus anticoagulant-hypoprothrombinemia syndrome", AMERICAN JOURNAL OF MEDICINE, 83, pp.563~566 (1987)
- Lillquist KB, "The absence of Factor II in a child with systemic lupus erythematosus", Acta Paediatrica Scandinavica, 67, pp.533~536 (1978)
