• Title/Summary/Keyword: Lupus anticoagulant

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Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome

  • Kim, Ji Soo;Kim, Min Jae;Bae, E. Young;Jeong, Dae Chul
    • Clinical and Experimental Pediatrics
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    • v.57 no.4
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    • pp.202-205
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    • 2014
  • Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids. Here, we report a case of LAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She was referred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPS that presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activated partial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment included massive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However, she died due to uncontrolled pulmonary hemorrhage.

Pulmonary Thromboendarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a Patient with Antiphospholipid Syndrome and Systemic Lupus Erythematosus (항인지질 증후군과 전신성 홍반성 루푸스 환자에게 발생된 만성 폐혈전색전성 폐동맥 고혈압에 대해 시행한 혈전내막제거술)

  • Kang, Pil-Je;Kim, Jeong-Won;Lee, Jae-Won
    • Journal of Chest Surgery
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    • v.40 no.12
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    • pp.867-870
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    • 2007
  • Antiphospholipid syndrome (APS) is defined as venous and/or arterial thromboses, recurrent fetal losses, thrombocytopenia in combination with repeatedly positive tests for the lupus anticoagulant (LAC), and anticardiolipin antibodies (aCL). The pulmonary manifestation is APS are relatively rare. We report a rare case of antiphopholipid syndrome with systemic lupus erythematosus in a patient who presented with pulmonary hypertension secondary to a chronic pulmonary thromboembolism. A bilateral thromboendarterectomy was performed satisfactorily and the incision was extended to the left intrapleural pulmonary artery.

Inhibitory Mechanism of Blood Coagulation and in vivo Anticoagulant Activities of Polysaccharides Isolated from Codium fragile (청각으로부터 분리한 다당류의 혈액응고 저해기작 및 in vivo 항응고 활성)

  • Shim, Yun-Yong;An, Jeung-Hee;Cho, Won-Dai;Chun, Hyug;Kim, Kyung-Im;Cho, Hong-You;Yang, Han-Chul
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.31 no.5
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    • pp.917-923
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    • 2002
  • Inhibitory mechanism of the anticoagulant polysaccharide purified from Codium fragile was investigated. The anticoagulant compounds (Cf-30-IV-4-ii, CF-30-IV) prolonged the clotting time at both activated partial thrombo-plastin time (aPTT) and thrombin time (TT). The Inhibition factor assay of instrinsic coagulation pathway in the blood showed that the anticoagulant polysaccharide (CF-30-IV-4-ii) inhibited other factors such as Ⅷ, Ⅸ, Ⅵ and Ⅷ of the coagulation cascade, which did not affect the lupus anticoagulant AB activity. In the thrombin inhibition pattern the CF-30-IV-4-ii did not directly influence the fibrine formation mediated by thrombin but af-fected the anticoagulant activity through the activation of antithrombin III. Base on these result, the anticoaglant polysaccharide (CF-30-IV-4-ii) was considered to inhibit serine pretense involved in the blood coagulation cascade through the enhancing antithrombin III activity. The residual effects of anticoagulant activity and antithrombosis were tested with ICR mice. The anticoagulant polysaccharide (CF-30-W) kept its anticoagulant activitv for 6 hrs with 100% survival at a dose of 150 mg/kg in the antithromboisis test. The anticoagulant effect of CF-30-RF in ex vivo was proportional to the concentration of intravenously injected dose up to 100 mg/kg.

A Case of Primary Antiphospholipid Antibody Syndrome Showing Vegetation on the Mitral Valve Through Echocardiography (승모판에서 증식증(vegetation)이 확인된 원발성 항인지질 항체 증후군 1례)

  • Lee, Seung-Yeop;Park, Seung-Kwon;Yun, Sung-Hwan;Jung, Yun-Seok;Kim, Hyen-Jik;Lim, Sung-Hwan;Hah, Jung-Sang;Kim, Wook-Nyeun
    • Journal of Yeungnam Medical Science
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    • v.15 no.2
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    • pp.350-358
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    • 1998
  • Antiphospholipid antibody syndrome(APS) is a well-known clinical syndrome characterized by recurrent arterial or venous thromboses, recurrent fetal loss, thrombocytopenia, together with high titers of sustained anticardiolipin antibody(aCL) or lupus anticoagulant(LA). Although systemic lupus erythematosus(SLB) and APS may coexist, a high proportion of patients manifesting the APS do not suffer from classical lupus or other connective tissue disease. The patient has been defined as having a primary antiphospholipid antibody syndrome. We experienced one case of primary APS with recurrent fetal loss, recurrent cerebral infarctions, positive anticardiolipin antibody IgG and fluttering vegetation on the mitral valve, without other connective tissue diseases including SLE. Forty-three old female had 2 out of 11 criteria for the diagnosis of SLE, such as thrombocytopenia and positive antinuclear antibody, but did not meet whole criteria. The patient was treated with ticlopidine, and anticoagulant therapy was recommended.

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A Case of Lupus Nephritis with Positive Antiphospholipid Antibodies, Initially Detected Through Analysis of Urinary Mass Screening (학교신체검사에서 발견된 항인지질 항체 양성 낭창성 신염 1례)

  • Lee Taek-Jin;Choi Min Sook;Lee Young-Mock;Kim Ji-Hong;Kim Pyung-Kil;Jeong Hyeon-Joo
    • Childhood Kidney Diseases
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    • v.5 no.2
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    • pp.219-224
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    • 2001
  • Antiphospholipid syndrome is a thrombotic disorder characterized by the association of arterial and venous thrombosis with the antibodies directed toward phospholipids. The presence of these antibodies in systemic lupus erythematosus(SLE) has been shown to be related to several clinical and analytical alterations. We experienced one case of lupus nephritis with positive antiphospholipid antibodies in a 10-year-old girl whose chief complaint was persistent microscopic hematuria. We report this case with a brief review of related literatures. (J. Korean Soc Pediatr Nephrol 2001 ;5 : 219-24)

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Inhibition of Platelet Aggregation by Anti-thyroglobulin Monoclonal Antibodies (Thyroglobulin에 대한 단일클론 항체의 혈소판응집 저해 작용)

  • Shon Yun Hee;Kim Cheorl Ho;Jeon Byung Hun;Nam Kyung Soo
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.18 no.2
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    • pp.534-537
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    • 2004
  • We produced twelve monoclonal antibodies(mAbs) against thyroglobulin and characterized the bindig profiles. Among them, three mAbs(TN-1, TN-2 and TN-3) were further characterized their binding specificities. TN-2 had a potent lupus anticoagulant activity and potentiated the anticoagulant effect of venom phospholipase A₂. he anticoagulant mechanism of TN-2 was elongation of the partial thromboplastin time and binding to phosphatidylserine which may have a pivot role in blood coagulation. And TN-2 was cross-reacted with ss-DNA and ds-DNA and had a characteristic of autoantibody. These results suggest that TN-2 may provide a useful tool for studying the correlation between autoimmune thyroiditis and its therapeutic effect.

A Case of Recurrent Spontaneous Abortion Associated with Antiphospholipid Syndrome (항인지질증후군과 관련된 반복자연유산 1례)

  • Nam, Y.S.;Han, S.Y.;Choi, D.H.;Yoon, T.K.;Cha, K.Y.
    • Clinical and Experimental Reproductive Medicine
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    • v.25 no.3
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    • pp.277-280
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    • 1998
  • Antiphospholipid antibody is an immunoglobulin which may be of any class and which reacts with any phospholipid. For clinical use the definition of the term anti-phospholipid antibody is usually restricted to IgG and/or IgM antibody directed against the negatively charged phopholipids, cardiolipin, phosphatidyl inositol, phosphatidyl serine, or phosphatidic acid. The antigen of the serological test for syphilis is cardiolipin; negatively charged phopholipids are understood to be antigens to which lupus anticoagulants are directed. The term 'anticardiolipin' antibody syndrome, 'antiphospholipid' antibody syndrome, and 'lupus anticoagulant' syndrome are often, imprecisely, used interchangeably. We have experienced a case of recurrent spontaneous abortion with antiphospholipid antibody. So we report this case with a brief review of literatures.

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A Case of Primary Antiphospholipid Syndrome with Pulmonary Thromboembolism (폐색전증을 동반한 원발성 항인지질증후군 1예)

  • Lee, Jae Beom;Sim, Yun Su;Noh, Young Wook;Park, Hye Sung;Tae, Chung Hyun;Lim, So Yeon;Jun, Yoon Hee;Ryu, Yon Ju;Chun, Eun Mi;Lee, Jin Hwa;Chang, Jung Hyun;Moon, Jin Wook
    • Tuberculosis and Respiratory Diseases
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    • v.63 no.1
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    • pp.72-77
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    • 2007
  • Antiphospholipid syndrome (APS) causes recurrent thromboses and morbidity during pregnancy, including fetal loss. This malady is associated with the persistent presence of anticardiolipin antibody or lupus anticoagulant. The pulmonary manifestations of antiphospholipid syndrome include pulmonary thromboembolism, pulmonary hypertension, acute respiratory distress syndrome, etc. Pulmonary thromboembolism is often the initial manifestation of antiphospholipid syndrome and a timely diagnosis is critical due to the high mortality rate. We herein report on a 19-year-old man with pulmonary thromboembolism that was caused by primary antiphospholipid syndrome. He presented with blood-tinged sputum, fever and epigastric pain, and his chest computerized tomography showed pulmonary thromboembolism. The other possible causes of pulmonary thromboembolism were excluded and the diagnosis of primary antiphospholipid syndrome was confirmed by the lupus anticoagulant that was present on two occasions six weeks apart. We also discuss the nature and management of antiphospholipid syndrome, along with a brief review of the relevant literatures.

The Study of Antithrombin III Deficiency in Patients with Recurrent Spontaneous Abortion (반복자연유산 환자에서 Antithrombin III 결핍증에 대한 연구)

  • Nam, Yoon-Sung;Cha, Kwang-Yul;Kim, Nam-Keun;Kang, Myung-Seo;Oh, Do-Yeon
    • Clinical and Experimental Reproductive Medicine
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    • v.28 no.4
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    • pp.301-305
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    • 2001
  • Objective : To analyze the antithrombin II deficiency in patients with recurrent spontaneous abortion. Material and Method: The blood samples were tested by chromogenic assay to evaluate the activity of antithrombin III. Results: There was only one case of antithrombin III deficiency. This patient experienced one neonatal death after delivery and one FDIU (fetal death in utero). And also this patient showed a lupus anticoagulant and the prolongation of PTT. Conclusions: Women with recurrent miscarriage who have no obvious identified cause should consider hematologic screening. Antithrombin III deficiency could be a cause of recurrent spontaneous abortion. But the incidence is very rare in Korean patients.

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A Case of Trousseau's Syndrome with Catastrophic Course Triggered by an Intravenous Injection (한번의 말초정맥주사로 유발되어 파국적으로 진행된 Trousseau증후군 1예)

  • Moon, Ji-Yong;Kim, Sa-Il;Kwak, Hyun-Jung;Song, Soon-Young;Kim, In-Soon;Kim, Sang-Heon;Kim, Tae-Hyung;Sohn, Jang-Won;Yoon, Ho-Joo;Shin, Dong-Ho;Park, Sung-Soo
    • Tuberculosis and Respiratory Diseases
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    • v.71 no.2
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    • pp.134-138
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    • 2011
  • Trousseau's syndrome is an unexplained thrombotic event that precedes the diagnosis of an occult visceral malignancy or appears concomitantly with the tumor. Upper extremity deep vein thrombosis is prevalent in patients with a central venous catheter. Furthermore, a peripheral intravenous injection may cause upper extremity deep vein thrombosis as well. However, a deep vein thrombosis has not been reported in the form of Trousseau's syndrome with a catastrophic clinical course triggered by a single peripheral intravenous injection. A 48-year-old man presented with a swollen left arm on which he was given intravenous fluid at a local clinic due to flu symptoms. Contrast computed tomgraphy scans showed thromboses from the left distal brachial to the innominate vein. The patient developed multiple cerebral infarctions despite anticoagulation treatment. He was diagnosed with stomach cancer by endoscopic biopsy to evaluate melena and had a persistently positive lupus anticoagulant. After recurrent and multiple thromboembolic events occurred with treatment, he died on day 20.