Lupus anticoagulant-hypoprothrombinemia in healthy adult

정춘해,박치영 대한내과학회 2008 The Korean Journal of Internal Medicine Vol.23 No.3

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. 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.

루푸스항응고인자-저프로트롬빈혈증 증후군으로 발현한 전신홍반루푸스 1예

설연아,이주훈,박영서,고경남,임호준,서종진 대한소아혈액종양학회 2014 Clinical Pediatric Hematology-Oncology Vol.21 No.2

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.

HELLP증후군 및 임신자간증과 동반된 항인지질증후군 1예

김효정,배창황,박진희,김태원,김준태,김우건 한국지혈혈전학회 1997 한국지혈혈전학회지 Vol.4 No.1

전신성 홍반성 낭창 환자에서 항 cardiolipin항체와 루프스 항응고인자의 출현 빈도와 임상적 의의

강문수,윤종수,강천일,서영익,황윤근,이종명,김능수 慶北大學校 醫科大學 1994 慶北醫大誌 Vol.35 No.3

목적 : 전신성홍반성낭창으로 확진된 환자들을 대상으로 항 cardiolipin,항체와 루프스 항응고인자의 출현 빈도와 임상 증상, 검사 소견 및 치료약제와의 관계를 분석하고 임상적 의의를 조사하였다. 대상 및 방법 : 전신성홍반성낭창으로 확진된 21예를 대상으로 하였으며 임상 및 검사소견을 분석하였고 항 cardiolipin항체는 ELISA법으로 측정하였고 루프스 항응고인자는 Kaolin clotting time 및 Platelet neutralization procedure를 함께 시행하여 측정하였다. 결과 : 전신성홍반성낭창 21예에서 항 cardiolipn항체는 6예(28.5%)에서 양성이었으며, 루프스 항응고인자는 단 1예(4.7%)에서 양성이었다. 항 cardiolipin항체 양성군과 음성군 사이에 연령, 성별 및 질병기간은 차이가 없었으며 광 과민성, 레이노 현상, 혈관염, 근관절증상, 심폐계증상, 신장병변 등의 임상소견도 차이가 없었다. 자연유산의 경험은 양성군에서 2예(40%), 음성군에서 1예(12.5%)에서 있었으며, 혈전증은 양성군 1예에서만 발생하였다. 양성군과 음성군사이에 빈혈, 백혈구감소증, 혈소판감소증, prothrombin time의 연장, 항핵 항체, 항 dsDNA항체, 혈청 보체감소 등의 검사 소견은 통계학적으로 차이가 없었다. 항 cardiolipin항체 양성군과 음성군사이에 IgG와 IgA치는 차이가 없었으나 IgM과 IgE치는 양성군에서 음성군보다 통계학적으로 유의하게 높았다. (P<0.05) 결론 : 항 cardiolipin항체와 혈전중, 자연유산, 혈소판감소증 및 혈관염 사이에는 별다른 상관관계를 발견할 수 없었으며 항 cardiolipin항체 양성군의 IgM및 IgE치의 증가가 질병의 활성도에 따른 변화 때문으로 생각되나 항 cardiolipin항체와의 직접적인 상관관계가 있을 가능성도 배제할 수 없을 것으로 생각된다. 전신성홍반성낭창환자에서 항 cardiolipin항체 양성군의 임상적 의의에 대해서 논란의 여지가 있으나 앞으로 더 많은 환자를 대상으로 이들의 임상적 의미에 대한 연구가 필요할 것으로 생각되며 또한 cardiolipin 이외의 각종 인지질에 대한 연구가 요구된다고 사료된다. We evaluated the prevalence of anti-cardiolipin antibodies and lupus anticoagulant in 21 patients with systemic lupus erythematosus(SLE) and investigated whether the presence of anti-cardiolipin antibodies was associated with paticular clinical or laboratory features. The prevalence of anti-cardiolipin antibodies was 28.5% and that of lupus anticoagulant was 4.7% in patients with SLE. No statistically significant association could be found between anti-cardiolipin antibodies and recurrent abortion, thrombosis, thrombocytopenia, and false positive VDRL. But there were significant association between anti-cardiolipin antibodies and serum immunoglobulin M, immunoglobulin E levels. Also, Various therapeutic regimens, including corticosteroids or antimalarial agents had no effect on the presence of anti-cardiolipin antibodies. Further studies are needed to clarify the pathogenetic role and clinical significance of anticardiolipin antibody, lupus anticoagulant.

Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome

김지수,김민재,배이영,정대철 대한소아청소년과학회 2014 Clinical and Experimental Pediatrics (CEP) Vol.57 No.4

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is causedby the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effectof lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids,immunosuppressive agents, and immunoglobulins for managing the disease and controllinghemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia andcontrolling 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 ofLAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She wasreferred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPSthat presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activatedpartial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment includedmassive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However,she died due to uncontrolled pulmonary hemorrhage.

A lupus anticoagulant-hypoprothrombinemia syndrome patient with adenovirus infection who was accidentally diagnosed after trauma

Lee Do-Jin,최두영 조선대학교 의학연구원 2022 Medical Bilogical Science and Engineering Vol.5 No.2

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHS) is a rare disease primarily known to occur in young adults and children with autoimmune diseases such as systemic lupus erythematosus or viral infection. Here, we reported a 4-year-old girl hospitalized with epistaxis, hematemesis, and mental changes that occurred after a fall. Recently, she presented with abdominal pain and loose stools. Prothrombin time (PT) and activated partial thromboplastin time (APTT) were prolonged. After 1:1 mixing of normal plasma, prolonged APTT was not corrected. Moreover, the factor II level decreased, and the lupus anticoagulant titer was positive. The adenovirus was also positive in the stool real-time PCR. Because the bleeding symptoms in LAHS, accompanied with clinically common adenovirus infection, were mild and transient, these diagnoses could be difficult and must be suspected in these viral infections. The bleeding tendency spontaneously improved after supportive medical treatment.

Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome

Kim, Ji Soo,Kim, Min Jae,Bae, E. Young,Jeong, Dae Chul The Korean Pediatric Society 2014 Clinical and Experimental Pediatrics (CEP) Vol.57 No.4

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.

Lupus Anticoagulant-hypoprothrombinemia Syndrome that did not Progress to Systemic Lupus Erythematosus: A Case Report

황영은,박흥근,염정숙,박지숙,박은실,서지현,임재영,박찬후,우향옥,윤희상 대한소아혈액종양학회 2015 Clinical Pediatric Hematology-Oncology Vol.22 No.2

Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare disorder, and appears mostly in children. The primary concern is its potential development into systemic lupus erythematosus (SLE). A 5-year-old patient was hospitalized with multiple purpuric lesions. A markedly prolonged prothrombin time and activated partial thromboplastin time were observed and were not corrected after mixing with normal plasma. Decreased factor II activity was consistent with LA-HPS. Identifying risk factors that play an important role in the development of SLE in patients with LA-HPS is of importance. Based on the case described here, anti-double stranded (ds) DNA antibody and the Sapporo criteria for antiphospholipid syndrome are related to subsequent SLE development, whereas there is no correlation with the results of the lupus anticoagulant (LA) test. We recommend an early and serial examination of anti-ds DNA antibody and full evaluation of Sapporo criteria for the screening of patients with LA-HPS who may progress to SLE.

Case Reports : Lupus anticoagulant-hypoprothrombinemia in healthy adult

Choon Hae Chung,Chi Young Park 대한내과학회 2008 The Korean Journal of Internal Medicine Vol.23 No.3

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.

육안적 혈뇨로 발현한 루푸스항응고인자-저프로트롬빈혈증 증후군

이재희 조선대학교 의학연구원 2016 The Medical Journal of Chosun University Vol.41 No.1

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHS) is a rare disease associated with lupus anticoagulant and factor II deficiency. LAHS is usually associated with autoimmune disease or with transient viral infection. A 27-month-old boy had a gross hematuria. Prothrombin time (PT) and activated partial thromboplastin time (aPTT) were markedly prolonged. The aPTT was not corrected after mixing with normal plasma. The level of factor II was decreased. Diluted Russell's viper venom test demonstrated the lupus anticoagulant. He was diagnosed with LAHS. His clinical symptom was improving without medical treatment. Serial laboratory studies showed that PT, aPTT, and factor II level were normalized.