폐색전증의 임상양상은 무증상에서 쇽 및 사망에 이르기까지 매우 다양 한 형태로 나타날 수 있다. 임상적으로 급성 폐색전증을 의심을 하는 것 은 특이적이지 않은데(Table 1), 증상과 소견이 폐색전증이 있거나 없는 환 자에서 유사하기 때문이다. 이러한 결과는 19개의 연구의 메타분석에서도 유사했고, 임상적인 판단으로 급성 폐색전증을 진단하는데 민감도는 85%, 특이도는 51%로 나타났다. 따라서 임상적으로 급성 폐색전증이 의심되면 추가적인 검사가 필요하다. 10% 이하로 비교적 드물지만 일시적 또는 지속 되는 심방세동, 실신 전 단계, 실신, 그리고 쇼크 등의 증상으로 나타나기 도 한다. 호흡곤란은 수초에서 수분 이내에 갑자기 발생하기도 하며, 고 령에서는 폐색전증이 발생해도 호흡곤란을 호소하는 경우가 낮다. 폐동맥 의 근위부에 색전증이 발생할수록 호흡곤란을 더 호소하는 경향이 있다. 10%의 환자에서는 원위부의 폐색전증으로 인한 폐경색(pulmonary infarction)으로 흉막 통증이나 객혈이 발생하기도 한다. 후향적 연구에서는 실신은 10% 이하로 나타나며, 반대로, 실신이 있는 사람들 중에는 폐색전증의 빈도가 2-17%였다. Review Article
{"title":"Diagnosis of the Adult with Suspected Acute Pulmonary Embolism","authors":"Won-Seok Choi","doi":"10.14345/ceth.21001","DOIUrl":"https://doi.org/10.14345/ceth.21001","url":null,"abstract":"폐색전증의 임상양상은 무증상에서 쇽 및 사망에 이르기까지 매우 다양 한 형태로 나타날 수 있다. 임상적으로 급성 폐색전증을 의심을 하는 것 은 특이적이지 않은데(Table 1), 증상과 소견이 폐색전증이 있거나 없는 환 자에서 유사하기 때문이다. 이러한 결과는 19개의 연구의 메타분석에서도 유사했고, 임상적인 판단으로 급성 폐색전증을 진단하는데 민감도는 85%, 특이도는 51%로 나타났다. 따라서 임상적으로 급성 폐색전증이 의심되면 추가적인 검사가 필요하다. 10% 이하로 비교적 드물지만 일시적 또는 지속 되는 심방세동, 실신 전 단계, 실신, 그리고 쇼크 등의 증상으로 나타나기 도 한다. 호흡곤란은 수초에서 수분 이내에 갑자기 발생하기도 하며, 고 령에서는 폐색전증이 발생해도 호흡곤란을 호소하는 경우가 낮다. 폐동맥 의 근위부에 색전증이 발생할수록 호흡곤란을 더 호소하는 경향이 있다. 10%의 환자에서는 원위부의 폐색전증으로 인한 폐경색(pulmonary infarction)으로 흉막 통증이나 객혈이 발생하기도 한다. 후향적 연구에서는 실신은 10% 이하로 나타나며, 반대로, 실신이 있는 사람들 중에는 폐색전증의 빈도가 2-17%였다. Review Article","PeriodicalId":249962,"journal":{"name":"Clinical & Experimental Thrombosis and Hemostasis","volume":"186 5 Suppl Nature 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132118341","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hyun-Ki Kim, Sang Hyuk Park, J. Lim, Joseph Jeong, S. Lee
Storage conditions, such as storage time and temperature, can affect the stability of coagulation factors and influence the results of coagulation assays. Therefore, a clinical laboratory should determine the allowable time interval between collection of the specimen and testing of the sample stored at a certain temperature. The Clinical and Laboratory Standards Institute (CLSI) guidelines (H21-A5) recommend that whole blood samples or plasma samples stored at room temperature for routine hemostasis tests or determination of coagulation factors should be analyzed within 4 hr after sample collection, with the exception of prothrombin time testing with stability up to 24 hr. However, there have been some studies suggesting samples stored for prolonged time periods are acceptable for reliable testing. For example, there were no clinically relevant changes in prothrombin time (PT) test results with up to a 24–48 hr delay. These data are practically important, because if a longer storage time were acceptable, resampling for additional coagulation testing and rejection of specimens due to prolonged delivery could be reduced. However, there are some differences between studies according to the storage conditions, testing method, and criteria for acceptability. Most studies have dealt with unspun blood samples (focusing on time from sample collection to delivery to the laboratory) or separated plasma. Therefore, these study data are not applicable to additional tests of samples with prolonged storage, considering most clinical samples are stored in the primary collection tube after initial testing. Additionally, there are few studies that include testing for fibrinogen degradation product (FDP) and coagulation inhibitors. This study aimed to investigate the stability of coagulation tests after storage of centrifuged samples in the primary collection tube with plasma remaining on top of the cells at room temperature for different time periods and to evaluate whether a longer storage period is acceptable compared with current CLSI guidelines. In this study, along with routine coagulation tests such as activated partial thromboplastin time (aPTT), prothrombin time (PT), fibrinogen, D-dimer, coagulation factors VIII (FVIII)/IX (FIX)/XI (FXI)/XII (FXII)/II (FII)/V (FV)/VII (FVII)/X (FX), and von Willebrand factor antigen (vWF antigen) and activity (vWF activity), tests such as antithrombin III (ATIII), FDP, dilute Russell’s viper venom time screening (dRVVT screen) and confirmation (confirm) were conducted.
{"title":"Validation of Pre-analytical Stability of Specimens Requested for Various Routine Coagulation Tests","authors":"Hyun-Ki Kim, Sang Hyuk Park, J. Lim, Joseph Jeong, S. Lee","doi":"10.14345/ceth.21002","DOIUrl":"https://doi.org/10.14345/ceth.21002","url":null,"abstract":"Storage conditions, such as storage time and temperature, can affect the stability of coagulation factors and influence the results of coagulation assays. Therefore, a clinical laboratory should determine the allowable time interval between collection of the specimen and testing of the sample stored at a certain temperature. The Clinical and Laboratory Standards Institute (CLSI) guidelines (H21-A5) recommend that whole blood samples or plasma samples stored at room temperature for routine hemostasis tests or determination of coagulation factors should be analyzed within 4 hr after sample collection, with the exception of prothrombin time testing with stability up to 24 hr. However, there have been some studies suggesting samples stored for prolonged time periods are acceptable for reliable testing. For example, there were no clinically relevant changes in prothrombin time (PT) test results with up to a 24–48 hr delay. These data are practically important, because if a longer storage time were acceptable, resampling for additional coagulation testing and rejection of specimens due to prolonged delivery could be reduced. However, there are some differences between studies according to the storage conditions, testing method, and criteria for acceptability. Most studies have dealt with unspun blood samples (focusing on time from sample collection to delivery to the laboratory) or separated plasma. Therefore, these study data are not applicable to additional tests of samples with prolonged storage, considering most clinical samples are stored in the primary collection tube after initial testing. Additionally, there are few studies that include testing for fibrinogen degradation product (FDP) and coagulation inhibitors. This study aimed to investigate the stability of coagulation tests after storage of centrifuged samples in the primary collection tube with plasma remaining on top of the cells at room temperature for different time periods and to evaluate whether a longer storage period is acceptable compared with current CLSI guidelines. In this study, along with routine coagulation tests such as activated partial thromboplastin time (aPTT), prothrombin time (PT), fibrinogen, D-dimer, coagulation factors VIII (FVIII)/IX (FIX)/XI (FXI)/XII (FXII)/II (FII)/V (FV)/VII (FVII)/X (FX), and von Willebrand factor antigen (vWF antigen) and activity (vWF activity), tests such as antithrombin III (ATIII), FDP, dilute Russell’s viper venom time screening (dRVVT screen) and confirmation (confirm) were conducted.","PeriodicalId":249962,"journal":{"name":"Clinical & Experimental Thrombosis and Hemostasis","volume":"12 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123792069","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
H. Hwang, J. H. Lee, Junshik Hong, Sang-A Kim, Yang-Ki Kim, M. Kim, S. Bang
Venous thromboembolism (VTE) is a significant cause of morbidity and mortality in patients with cancer irrespective of cancer stage. Two successive nationwide population-based epidemiologic studies conducted between 2004 and 2013 using the Korean Health Insurance Review and Assessment Service (HIRA) databases showed a gradual increase in annual ageand sex-standardized incidence (ASR) of VTE over the decade, even though the incidence rates in Republic of Korea are lower than those in Western countries. Interestingly, recent studies have shown that, unlike the incidence rates, the recurrence rates of VTE in Asia are comparable to those in Western countries. Therefore, the recurrence rate of cancer-associated VTE (CAT) in Asia can be expected to be as high as that reported in a Western population but has not yet been reported. This study aimed to evaluate the recurrence rates of anticoagulant use in patients with CAT vs. non-cancer-associated thrombosis VTE (NCAT) using Republic of Korea HIRA data collected between 2009 and 2013. Methods
{"title":"Recurrence of Cancer-associated Venous Thromboembolism between 2009 and 2013: A Nationwide Korean Study","authors":"H. Hwang, J. H. Lee, Junshik Hong, Sang-A Kim, Yang-Ki Kim, M. Kim, S. Bang","doi":"10.14345/ceth.21003","DOIUrl":"https://doi.org/10.14345/ceth.21003","url":null,"abstract":"Venous thromboembolism (VTE) is a significant cause of morbidity and mortality in patients with cancer irrespective of cancer stage. Two successive nationwide population-based epidemiologic studies conducted between 2004 and 2013 using the Korean Health Insurance Review and Assessment Service (HIRA) databases showed a gradual increase in annual ageand sex-standardized incidence (ASR) of VTE over the decade, even though the incidence rates in Republic of Korea are lower than those in Western countries. Interestingly, recent studies have shown that, unlike the incidence rates, the recurrence rates of VTE in Asia are comparable to those in Western countries. Therefore, the recurrence rate of cancer-associated VTE (CAT) in Asia can be expected to be as high as that reported in a Western population but has not yet been reported. This study aimed to evaluate the recurrence rates of anticoagulant use in patients with CAT vs. non-cancer-associated thrombosis VTE (NCAT) using Republic of Korea HIRA data collected between 2009 and 2013. Methods","PeriodicalId":249962,"journal":{"name":"Clinical & Experimental Thrombosis and Hemostasis","volume":"127 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133831249","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Jae-lim Choi, Ri-Young Goh, Seong-Hoon Yun, Joo-In Park, Jin-Yeong Han
Platelets are anuclear cells released from megakaryocytes, the rare myeloid cells that undergo multiple rounds of endomitosis before releasing platelets. Platelets, known as the mediator of thrombosis, are emerging as cellular mediators of type II diabetes, atherosclerosis, cancer cell metastasis, and immune responses. Despite the lack of a nucleus, they express a large number of transcription factors including peroxisome proliferator-activated receptor (PPAR). PPAR needs ligand-binding to be activated. PPAR, a ligand-activated transcription factor, consists of 3 isoforms: α, β/δ, γ, and requires heterodimerization with retinoid X receptor to modulate transcription of target genes. PPARγ is involved in glucose metabolism, inflammation, and differentiation and functions of adipose tissue. Recent studies have reported that the PPARγ inhibits platelet activation and aggregation. Also, studies have reported the PPARγ and ligand of PPARγ as the factor influences the hematopoietic system. They could affect the roles of erythroid, myeloid, monocytic, and lymphocytic cell function during an immune response, and modulate proliferation and maturation of erythroid progenitor cells. The endogenous 15-deoxy-Δ prostaglandin J2 (15d-PGJ2) is a ligand of PPARγ. Biologically, 15d-PGJ2 is the most active metabolite of prostaglandin D2. Fucoidans, fucose-containing sulfated polysaccharides, are constituents of brown seaweed and some marine invertebrates. Extensive studies have reported their various biological effects including immunostimulation, anti-tumor, antiviral, antithrombotic and anticoagulant activities. By far the anticoagulant and antithrombotic actions of fucoidans are the most widely recognized bioactivities, but the basis for these activities is not completely understood. We are interested in the influences of 15d-PGJ2 and fucoidans to the megakaryocytic differentiation and the platelet production, as well as the influences of 15d-PGJ2 and fucoidans to the thrombosis and coagulation system. Due to their antithrombotic and anticoagulant activities, they have the probability of the application as the novel therapeutic agents to cardiovascular disease. Also, if they have the abilities inducing the production of platelet, they could be the stable solution of platelet supply to the thrombocytopenic patients. In this study, we evaluated the effects of 15d-PGJ2 and fucoidans on megakaryocytes and platelets with diverse methods from protein to the molecular level. This study aims to determine 1) whether the 15d-PGJ2 and fucoidans encourage the megakaryocytes to produce platelets actively; 2) whether the 15d-PGJ2 and fucoidans possess the anticoagulant and antithrombotic actions, and 3) whether specific factors, such as prostaglandins or genes, explain the mechanism of how the 15d-PGJ2 and fucoidans influence differentiation and function of the platelets. To search these factors, we also included PPARα, which activates fatty acid catabolism and stimulates gluc
{"title":"Effects of Peroxisome Proliferator-Activated Receptor Ligand and Brown Seaweed Based Compound on Megakaryocyte","authors":"Jae-lim Choi, Ri-Young Goh, Seong-Hoon Yun, Joo-In Park, Jin-Yeong Han","doi":"10.14345/ceth.19001","DOIUrl":"https://doi.org/10.14345/ceth.19001","url":null,"abstract":"Platelets are anuclear cells released from megakaryocytes, the rare myeloid cells that undergo multiple rounds of endomitosis before releasing platelets. Platelets, known as the mediator of thrombosis, are emerging as cellular mediators of type II diabetes, atherosclerosis, cancer cell metastasis, and immune responses. Despite the lack of a nucleus, they express a large number of transcription factors including peroxisome proliferator-activated receptor (PPAR). PPAR needs ligand-binding to be activated. PPAR, a ligand-activated transcription factor, consists of 3 isoforms: α, β/δ, γ, and requires heterodimerization with retinoid X receptor to modulate transcription of target genes. PPARγ is involved in glucose metabolism, inflammation, and differentiation and functions of adipose tissue. Recent studies have reported that the PPARγ inhibits platelet activation and aggregation. Also, studies have reported the PPARγ and ligand of PPARγ as the factor influences the hematopoietic system. They could affect the roles of erythroid, myeloid, monocytic, and lymphocytic cell function during an immune response, and modulate proliferation and maturation of erythroid progenitor cells. The endogenous 15-deoxy-Δ prostaglandin J2 (15d-PGJ2) is a ligand of PPARγ. Biologically, 15d-PGJ2 is the most active metabolite of prostaglandin D2. Fucoidans, fucose-containing sulfated polysaccharides, are constituents of brown seaweed and some marine invertebrates. Extensive studies have reported their various biological effects including immunostimulation, anti-tumor, antiviral, antithrombotic and anticoagulant activities. By far the anticoagulant and antithrombotic actions of fucoidans are the most widely recognized bioactivities, but the basis for these activities is not completely understood. We are interested in the influences of 15d-PGJ2 and fucoidans to the megakaryocytic differentiation and the platelet production, as well as the influences of 15d-PGJ2 and fucoidans to the thrombosis and coagulation system. Due to their antithrombotic and anticoagulant activities, they have the probability of the application as the novel therapeutic agents to cardiovascular disease. Also, if they have the abilities inducing the production of platelet, they could be the stable solution of platelet supply to the thrombocytopenic patients. In this study, we evaluated the effects of 15d-PGJ2 and fucoidans on megakaryocytes and platelets with diverse methods from protein to the molecular level. This study aims to determine 1) whether the 15d-PGJ2 and fucoidans encourage the megakaryocytes to produce platelets actively; 2) whether the 15d-PGJ2 and fucoidans possess the anticoagulant and antithrombotic actions, and 3) whether specific factors, such as prostaglandins or genes, explain the mechanism of how the 15d-PGJ2 and fucoidans influence differentiation and function of the platelets. To search these factors, we also included PPARα, which activates fatty acid catabolism and stimulates gluc","PeriodicalId":249962,"journal":{"name":"Clinical & Experimental Thrombosis and Hemostasis","volume":"20 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125258875","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
번 응고인자가 억제되고 고갈되어 출혈과 관련된 합병증이 발생하는 매우 드문 질환이다. 종종 생명을 위협하는 치명적인 출혈 합병증이 발생하는 것으로 알려져 있어, 관련된 증상이 동반된 환자에서 의심을 하여 조기에 진단을 하는 것이 매우 중요하다. 후천성혈우병 A는 100만 명 중 1년에 0.2-1.48명이 발생하는 것으로 보 고되고 있다. 20-30대 여성에서는 임신이나 자가면역성 질환과 관련하여 종종 발생하나, 대부분은 65세 이상의 고령(중앙값 64-78세)에서 남녀에 모두 비슷한 비율로 발생한다. 소아에서는 매우 드문 것으로 알려져 있 다. 자가면역성 질환(전신홍반루푸스, 류마티스관절염 등), 악성종양, 임신 (대부분 첫 번째 임신에서 발생하며, 출산 후 1-4개월에 발생함. 자가항체 가 태반을 통과하므로 태아에 출혈 위험이 있음), 감염, 약제 등 후천성 혈우병 A 발생에 관련된 원인이 있는 경우가 50% 정도 되며, 나머지 50% 에서는 원인불명(idiopathic)으로 밝혀진 관련된 질환 없이 발생하는 것으 로 알려져 있다. 비록 약 30% 정도에서는 출혈이 심하지 않아 출혈에 대 한 지혈치료가 필요 없으나, 최근의 대규모 보고에 따르면 94.6%에서는 출 혈성 임상양상이 동반되었고, 77%의 자연출혈과 70%의 심각한 출혈(혈 색소 < 8 g/dL 또는 혈색소 >2 g/dL 감소로 정의됨)이 관찰되었다. 선천성 혈우병과는 나타나는 임상양상에 차이가 있다. 선천성혈우병에서 주로 관 찰되는 관절강내출혈(hemarthrosis)은 드물고, 후천성혈우병 A에서는 피 하출혈이 흔하게 관찰된다(> 80%). 근육내 출혈이나 위장관계 출혈도 종 종 관찰된다(Table 1). 사망률은 특히 65세 이상의 고령이나 기저 악성 종양을 동반한 경우에는 20% 이상으로 보고되고 있다. 기저질환 관련 사 망이 많고, 출혈에 의한 사망은 5-10% 정도로 알려져 있다(이전 연구에서 3.2%와 9.1%로 보고됨). 후천성혈우병 A는 진단의 지연으로 인하여 치명 적인 출혈에 의한 사망이 발생할 수 있으며, 진단과 적절한 초기 조치가 되
是一种非常罕见的疾病，它会抑制和枯竭，从而引发出血相关的并发症。据悉，经常会发生危及生命的致命出血并发症，因此对伴有相关症状的患者进行怀疑并早期诊断非常重要。获得性血友病A在100万名中，1年发生0.2-1.48名。20-30岁的女性经常发生与怀孕或自身免疫性疾病相关的疾病，但大部分在65岁以上的高龄(中央值64-78岁)男女均发生相似的情况。据悉，在少儿中非常罕见。自身免疫性疾病(全身红斑狼疮、风湿性关节炎等)、恶性肿瘤、妊娠(多发生在第一次妊娠，分娩后1-4个月发生。由于自身抗体会通过胎盘，因此胎儿有出血的危险)、感染、药剂等后天性血友病A发生相关的原因占50%左右，剩下的50%是在没有原因不明(idiopathic)的相关疾病的情况下发生。虽然约在30%左右,出血并不严重,对出血的止血不需要治疗,但最近的大规模报告显示,在94.6%,伴有血性的临床表现,77%的自然失血性和70%的严重出血(色素< 8 g / dl或血红蛋白减少,随着定义2 g / dl)观察了。与先天性血友病在临床表现上有差异。先天性血友病中常见的关节腔内出血(hemarthrosis)很少，获得性血友病A中常见的是皮下出血(> 80%)。肌肉内出血和胃肠关系出血也可以观察到肿块(Table 1)，特别是65岁以上的高龄或伴随基底恶性肿瘤的死亡率为20%以上。与基底疾病相关的死亡人数较多，因出血而死亡的人数约为5-10%(在之前的研究中报告为3.2%和9.1%)。获得性血友病A可能因诊断延迟导致致命出血而死亡，诊断和适当的初期措施;
{"title":"Acquired Hemophilia A","authors":"Jin Seok Kim","doi":"10.14345/ceth.19003","DOIUrl":"https://doi.org/10.14345/ceth.19003","url":null,"abstract":"번 응고인자가 억제되고 고갈되어 출혈과 관련된 합병증이 발생하는 매우 드문 질환이다. 종종 생명을 위협하는 치명적인 출혈 합병증이 발생하는 것으로 알려져 있어, 관련된 증상이 동반된 환자에서 의심을 하여 조기에 진단을 하는 것이 매우 중요하다. 후천성혈우병 A는 100만 명 중 1년에 0.2-1.48명이 발생하는 것으로 보 고되고 있다. 20-30대 여성에서는 임신이나 자가면역성 질환과 관련하여 종종 발생하나, 대부분은 65세 이상의 고령(중앙값 64-78세)에서 남녀에 모두 비슷한 비율로 발생한다. 소아에서는 매우 드문 것으로 알려져 있 다. 자가면역성 질환(전신홍반루푸스, 류마티스관절염 등), 악성종양, 임신 (대부분 첫 번째 임신에서 발생하며, 출산 후 1-4개월에 발생함. 자가항체 가 태반을 통과하므로 태아에 출혈 위험이 있음), 감염, 약제 등 후천성 혈우병 A 발생에 관련된 원인이 있는 경우가 50% 정도 되며, 나머지 50% 에서는 원인불명(idiopathic)으로 밝혀진 관련된 질환 없이 발생하는 것으 로 알려져 있다. 비록 약 30% 정도에서는 출혈이 심하지 않아 출혈에 대 한 지혈치료가 필요 없으나, 최근의 대규모 보고에 따르면 94.6%에서는 출 혈성 임상양상이 동반되었고, 77%의 자연출혈과 70%의 심각한 출혈(혈 색소 < 8 g/dL 또는 혈색소 >2 g/dL 감소로 정의됨)이 관찰되었다. 선천성 혈우병과는 나타나는 임상양상에 차이가 있다. 선천성혈우병에서 주로 관 찰되는 관절강내출혈(hemarthrosis)은 드물고, 후천성혈우병 A에서는 피 하출혈이 흔하게 관찰된다(> 80%). 근육내 출혈이나 위장관계 출혈도 종 종 관찰된다(Table 1). 사망률은 특히 65세 이상의 고령이나 기저 악성 종양을 동반한 경우에는 20% 이상으로 보고되고 있다. 기저질환 관련 사 망이 많고, 출혈에 의한 사망은 5-10% 정도로 알려져 있다(이전 연구에서 3.2%와 9.1%로 보고됨). 후천성혈우병 A는 진단의 지연으로 인하여 치명 적인 출혈에 의한 사망이 발생할 수 있으며, 진단과 적절한 초기 조치가 되","PeriodicalId":249962,"journal":{"name":"Clinical & Experimental Thrombosis and Hemostasis","volume":"80 2 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130720207","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
H. Hwang, M. Kim, G. Lim, S. Koo, B. Lee, Jun Yong Chang, Ki-Up Kim, R. Park, J. Lee, S. Uh, Yang-Ki Kim
The standard regimen with vitamin K antagonist (VKA) and lowmolecular-weight heparin (LMWH) was the main therapeutic option in patients with venous thromboembolisms (VTE) until the launch of direct oral anticoagulants (DOAC) in 2013 in South Korea. The ability of health-care provider to make appropriate dosage of VKA (e.g., warfarin) and follow-up decisions can have a major impact on therapeutic effectiveness and safety such as recurrent VTE and bleeding events, respectively. The quality of anticoagulation can be assessed by time in therapeutic range (TTR). A strong relationship between TTR and bleeding or thromboembolic rates has been observed across studies. However, TTR in real clinical practice was lower than 57% to 64% in clinical trials. The objective of this study was to investigate the quality of anticoagulation with TTR in patients with VTE in a single tertiary hospital in Korea.
{"title":"Quality Assessment of Oral Anticoagulation by Time in Therapeutic Range in Patients with Venous Thromboembolism in Korea","authors":"H. Hwang, M. Kim, G. Lim, S. Koo, B. Lee, Jun Yong Chang, Ki-Up Kim, R. Park, J. Lee, S. Uh, Yang-Ki Kim","doi":"10.14345/ceth.19002","DOIUrl":"https://doi.org/10.14345/ceth.19002","url":null,"abstract":"The standard regimen with vitamin K antagonist (VKA) and lowmolecular-weight heparin (LMWH) was the main therapeutic option in patients with venous thromboembolisms (VTE) until the launch of direct oral anticoagulants (DOAC) in 2013 in South Korea. The ability of health-care provider to make appropriate dosage of VKA (e.g., warfarin) and follow-up decisions can have a major impact on therapeutic effectiveness and safety such as recurrent VTE and bleeding events, respectively. The quality of anticoagulation can be assessed by time in therapeutic range (TTR). A strong relationship between TTR and bleeding or thromboembolic rates has been observed across studies. However, TTR in real clinical practice was lower than 57% to 64% in clinical trials. The objective of this study was to investigate the quality of anticoagulation with TTR in patients with VTE in a single tertiary hospital in Korea.","PeriodicalId":249962,"journal":{"name":"Clinical & Experimental Thrombosis and Hemostasis","volume":"14 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124932370","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
항트롬빈(antithrombin III), C단백(protein C), S단백(protein S)은 모두 간에서 합성되는 비타민 K-의존 자연항응고제(natural anticoagulant)로 서 선천적이든 후천적이든 결핍 시 혈전성향을 보일 수 있다. 이들 단백이 결핍되면 임상적으로 주로 심부정맥혈전증(deep vein thrombosis, DVT) 또는 폐색전증(pulmonary embolism, PE)이 발생할 수 있다. 서양에서 C 단백 결핍은 200-500명 중 1명, S단백 결핍은 500명 중 1명, 항트롬빈 결핍 은 2,000-5,000명 중 1명 정도로 보고된다. 항트롬빈 결핍이 가장 드물지 만 환자의 50% 정도까지에서 일생 중 혈전을 경험하게 된다. 항트롬빈은 432개의 아미노산으로 이루어진 비타민 K-의존 당단백으 로서 3개의 이황화결합을 가지고 있으며 4개의 당화(glycosylation) 부위 가 있다. 주된 형태인 α-트롬빈은 4개의 당화부위가 모두 올리고당(oligosaccharide)과 결합되어 있다. 항트롬빈은 혈액응고계에서 주로 FIIa와 FXa를 억제하지만 FVIIa, FIXa, FXIa 등도 억제한다. 특히, 헤파린이 있으 면 2,000-4,000배 정도 그 억제 반응이 증강된다. C단백은 경쇄와 중쇄가 이황화결합으로 연결되어 이루어진 비타민 K-의존 당단백이다. 혈액 내의 C단백은 트롬빈이 내피세포 표면의 트롬보모듈린에 결합하 면 활성화된다. 활성화 C단백은 인지질, 칼슘, S단백의 도움을 받아 FVa와 FVIIIa을 불활성화시킴으로써 응고계를 조절하는 역할을 한다. 단백 S는 635개의 아미노산으로 이루어진 비타민 K 의존 당단백으로 단백 C의 인 지질에 대한 친화력을 증강시켜 FVa와 FVIIIa를 불활성화시켜 혈액응고 를 억제하는 보조인자로 작용한다. 혈중 단백 S의 약 60%는 C4b-결합단 백(C4b-binding protein, C4b-BP)과 결합하여 존재하고 나머지 40% 정도 만 유리형으로 존재하는데 단백 C의 보조인자로서의 역할은 유리형이 가 지고 있다). 항트롬빈, C단백, S단백의 정확한 측정은 정맥혈전색전증 환자들을 진 단하고 평가하는 데 도움이 된다. 이 연구에서는 항트롬빈, C단백, 유리형 Original Article
{"title":"Performance Evaluation of Innovance Antithrombin, Berichrom Protein C and Innovance Free Protein S Assays Using the Automated Coagulation Analyzer Sysmex CS-5100","authors":"W. Shin, Min Sook Seo, R. Park","doi":"10.14345/CETH.18003","DOIUrl":"https://doi.org/10.14345/CETH.18003","url":null,"abstract":"항트롬빈(antithrombin III), C단백(protein C), S단백(protein S)은 모두 간에서 합성되는 비타민 K-의존 자연항응고제(natural anticoagulant)로 서 선천적이든 후천적이든 결핍 시 혈전성향을 보일 수 있다. 이들 단백이 결핍되면 임상적으로 주로 심부정맥혈전증(deep vein thrombosis, DVT) 또는 폐색전증(pulmonary embolism, PE)이 발생할 수 있다. 서양에서 C 단백 결핍은 200-500명 중 1명, S단백 결핍은 500명 중 1명, 항트롬빈 결핍 은 2,000-5,000명 중 1명 정도로 보고된다. 항트롬빈 결핍이 가장 드물지 만 환자의 50% 정도까지에서 일생 중 혈전을 경험하게 된다. 항트롬빈은 432개의 아미노산으로 이루어진 비타민 K-의존 당단백으 로서 3개의 이황화결합을 가지고 있으며 4개의 당화(glycosylation) 부위 가 있다. 주된 형태인 α-트롬빈은 4개의 당화부위가 모두 올리고당(oligosaccharide)과 결합되어 있다. 항트롬빈은 혈액응고계에서 주로 FIIa와 FXa를 억제하지만 FVIIa, FIXa, FXIa 등도 억제한다. 특히, 헤파린이 있으 면 2,000-4,000배 정도 그 억제 반응이 증강된다. C단백은 경쇄와 중쇄가 이황화결합으로 연결되어 이루어진 비타민 K-의존 당단백이다. 혈액 내의 C단백은 트롬빈이 내피세포 표면의 트롬보모듈린에 결합하 면 활성화된다. 활성화 C단백은 인지질, 칼슘, S단백의 도움을 받아 FVa와 FVIIIa을 불활성화시킴으로써 응고계를 조절하는 역할을 한다. 단백 S는 635개의 아미노산으로 이루어진 비타민 K 의존 당단백으로 단백 C의 인 지질에 대한 친화력을 증강시켜 FVa와 FVIIIa를 불활성화시켜 혈액응고 를 억제하는 보조인자로 작용한다. 혈중 단백 S의 약 60%는 C4b-결합단 백(C4b-binding protein, C4b-BP)과 결합하여 존재하고 나머지 40% 정도 만 유리형으로 존재하는데 단백 C의 보조인자로서의 역할은 유리형이 가 지고 있다). 항트롬빈, C단백, S단백의 정확한 측정은 정맥혈전색전증 환자들을 진 단하고 평가하는 데 도움이 된다. 이 연구에서는 항트롬빈, C단백, 유리형 Original Article","PeriodicalId":249962,"journal":{"name":"Clinical & Experimental Thrombosis and Hemostasis","volume":"39 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126165972","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
정맥혈전색전증(VTE)의 합병증으로는 혈전후증후군(post-thrombotic syndrome)과 만성혈전색전폐고혈압(chronic thromboembolic pulmonary hypertension, CTEPH)을 들 수 있다. 혈전후증후군은 만성 정맥 기 능 부전으로 통증, 부종을 특징으로 하며 궤양이 발생하기도 한다. 심부정 맥혈전(deep vein thrombosis, DVT)을 적절히 치료한 후에도 20-50%까 지 혈전후증후군이 발생한다는 보고가 있다. 혈전후증후군은 DVT의 가 장 흔한 합병증이면서, DVT 재발의 위험인자이다. CTEPH은 일반적 으로 폐색전증(pulmonary embolism, PE) 이후에 발생하는 것으로 알려 졌지만 약 25%에서는 폐색전의 병력이 없다. CTEPH은 우심실부전의 증 상인 호흡 곤란, 말초 부종, 실신 등을 특징으로 한다. PE 후 CTEPH 발생 률은 1-4%이다. 이러한 만성 합병증은 삶의 질을 떨어뜨리고, 의료비용 을 많이 증가시키게 된다. VTE가 처음 발생한 후에 위험 요소가 일시적인지 영구적인지 여부로 재발의 위험을 분류하기 시작했다. VTE가 일시적인 유발인자에 의해 발 생했다면, 치료 후 VTE 재발 가능성도 적지만, 전이성 암과 같은 지속적인 VTE 재발의 위험 요소가 있는 경우에는 재발 위험이 높을 것으로 분류한다. VTE 환자의 50% 이상은 위험 요소가 없이 발생한다. 위험 요소 없 는 unprovoked VTE가 처음 발생한 환자에서 치료를 종료한 후 재발 위험 Review Article
{"title":"The Optimal Duration and Selection of Anti-coagulants after First Episode of Unprovoked Venous Thromboembolism","authors":"W. Choi, Yang-Ki Kim","doi":"10.14345/CETH.18001","DOIUrl":"https://doi.org/10.14345/CETH.18001","url":null,"abstract":"정맥혈전색전증(VTE)의 합병증으로는 혈전후증후군(post-thrombotic syndrome)과 만성혈전색전폐고혈압(chronic thromboembolic pulmonary hypertension, CTEPH)을 들 수 있다. 혈전후증후군은 만성 정맥 기 능 부전으로 통증, 부종을 특징으로 하며 궤양이 발생하기도 한다. 심부정 맥혈전(deep vein thrombosis, DVT)을 적절히 치료한 후에도 20-50%까 지 혈전후증후군이 발생한다는 보고가 있다. 혈전후증후군은 DVT의 가 장 흔한 합병증이면서, DVT 재발의 위험인자이다. CTEPH은 일반적 으로 폐색전증(pulmonary embolism, PE) 이후에 발생하는 것으로 알려 졌지만 약 25%에서는 폐색전의 병력이 없다. CTEPH은 우심실부전의 증 상인 호흡 곤란, 말초 부종, 실신 등을 특징으로 한다. PE 후 CTEPH 발생 률은 1-4%이다. 이러한 만성 합병증은 삶의 질을 떨어뜨리고, 의료비용 을 많이 증가시키게 된다. VTE가 처음 발생한 후에 위험 요소가 일시적인지 영구적인지 여부로 재발의 위험을 분류하기 시작했다. VTE가 일시적인 유발인자에 의해 발 생했다면, 치료 후 VTE 재발 가능성도 적지만, 전이성 암과 같은 지속적인 VTE 재발의 위험 요소가 있는 경우에는 재발 위험이 높을 것으로 분류한다. VTE 환자의 50% 이상은 위험 요소가 없이 발생한다. 위험 요소 없 는 unprovoked VTE가 처음 발생한 환자에서 치료를 종료한 후 재발 위험 Review Article","PeriodicalId":249962,"journal":{"name":"Clinical & Experimental Thrombosis and Hemostasis","volume":"114 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"128153495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J. X. Li, Moo-Hyun Kim, Kai Song, L. Guo, Enze Jin, S. Choi, K. Lee
Warfarin, as a common oral anticoagulant, is used to prevent and treat thromboembolic diseases such as atrial fibrillation (AF), heart valve replacement, deep venous thrombosis, and pulmonary embolism. Warfarin has many disadvantages such as narrow therapeutic index and wide dose variation of interindividual response, and its stable dosage is influenced by a variety of factors. Thus, determining warfarin therapy is challenging. Several studies have shown that genetic polymorphisms affect warfarin pharmacodynamics, especially CYP2C9 and vitamin K epoxide reductase complex subunit 1 (VKORC1). One study demonstrated that the VKORC1 genotype can explain about 27% of warfarin dose variation, and the CYP2C9 genotype can explain about 7% of the warfarin dose variation in Asian patients. Combining this with other non-genetic factors, VKORC1 and CYP2C9 in warfarin pharmacokinetics and pharmacodynamics account for approximately 50% of inter-individual warfarin dose variation. However, half of the variation sources are still unknown. CYP4F2 is an enzyme that can catalyze multiple reactions and affect warfarin dose. CYP4F2 is a primary liver vitamin K1 oxidase that catalyzes the metabolism of vitamin K1 to hydroxy-vitamin K1 and removes vitamin K from the vitamin K cycle, which can lead to less vitamin K available for clotting factor activation. The physiologic role of CYP4F2 in the vitamin K/warfarin pathway is controversial. Some studies have shown that the CYP4F2 genotype can affect warfarin dose. Compared to wild-type patients, patients with CYP4F2 variants need higher warfarin dose. But some papers reported that the CYP4F2 genotype did not affect warfarin dose. In addition, the CYP4F2 gene influence on warfarin dosage has not been frequently tested in the Korean population. Therefore, we sought to determine if CYP4F2 could affect warfarin dosage in Korean patients with a variety of diseases.
{"title":"The Influence of CYP4F2 Polymorphisms on Warfarin Doses in Korean Patients with a Variety of Diseases","authors":"J. X. Li, Moo-Hyun Kim, Kai Song, L. Guo, Enze Jin, S. Choi, K. Lee","doi":"10.14345/CETH.18002","DOIUrl":"https://doi.org/10.14345/CETH.18002","url":null,"abstract":"Warfarin, as a common oral anticoagulant, is used to prevent and treat thromboembolic diseases such as atrial fibrillation (AF), heart valve replacement, deep venous thrombosis, and pulmonary embolism. Warfarin has many disadvantages such as narrow therapeutic index and wide dose variation of interindividual response, and its stable dosage is influenced by a variety of factors. Thus, determining warfarin therapy is challenging. Several studies have shown that genetic polymorphisms affect warfarin pharmacodynamics, especially CYP2C9 and vitamin K epoxide reductase complex subunit 1 (VKORC1). One study demonstrated that the VKORC1 genotype can explain about 27% of warfarin dose variation, and the CYP2C9 genotype can explain about 7% of the warfarin dose variation in Asian patients. Combining this with other non-genetic factors, VKORC1 and CYP2C9 in warfarin pharmacokinetics and pharmacodynamics account for approximately 50% of inter-individual warfarin dose variation. However, half of the variation sources are still unknown. CYP4F2 is an enzyme that can catalyze multiple reactions and affect warfarin dose. CYP4F2 is a primary liver vitamin K1 oxidase that catalyzes the metabolism of vitamin K1 to hydroxy-vitamin K1 and removes vitamin K from the vitamin K cycle, which can lead to less vitamin K available for clotting factor activation. The physiologic role of CYP4F2 in the vitamin K/warfarin pathway is controversial. Some studies have shown that the CYP4F2 genotype can affect warfarin dose. Compared to wild-type patients, patients with CYP4F2 variants need higher warfarin dose. But some papers reported that the CYP4F2 genotype did not affect warfarin dose. In addition, the CYP4F2 gene influence on warfarin dosage has not been frequently tested in the Korean population. Therefore, we sought to determine if CYP4F2 could affect warfarin dosage in Korean patients with a variety of diseases.","PeriodicalId":249962,"journal":{"name":"Clinical & Experimental Thrombosis and Hemostasis","volume":"264 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133634411","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Venous thromboembolism (VTE), including deep vein thrombosis (DVT) and pulmonary embolism (PE), is a major health concern and cause of mortality worldwide. Among the well-established risk factors for VTE, congestive heart failure (CHF) is a well-known major risk factor, especially in hospitalized patients. In CHF patients, the risk of VTE is increased because of multi-factorial causes including blood stasis due to decreased ejection fraction (EF), immobilization, endothelial dysfunction, and hemostatic abnormalities. Thus, VTE is approximately three times more likely to develop in CHF patients than in individuals without CHF. Most previous studies regarding VTE in CHF patients were conducted in Caucasian populations. The reported incidence of VTE in CHF patients has widely ranged from 1.63% to 59%. Recently, several epidemiologic studies in the Asian population demonstrated that VTE incidence is rapidly increasing, making VTE an important health concern in Asian countries. In this respect, we hypothesized that CHF is an important risk factor for VTE in Asian populations. To date, however, despite the clinical significance of CHF as a likely VTE risk factor, its contribution to VTE risk has not been established in an Asian population. Therefore, we conducted this nationwide epidemiologic study using the Korean Health Insurance Review and Assessment Service (HIRA) database to analyze the VTE prevalence in CHF patients. We also performed a retrospective observation study to investigate potential VTE risks in Korean CHF patients. Methods
{"title":"Prevalence and Risk Factor of Venous Thromboembolism in Korean Patients with Congestive Heart Failure","authors":"M. Jang, H. Kwon, M. Jeong, D. Oh","doi":"10.14345/CETH.18004","DOIUrl":"https://doi.org/10.14345/CETH.18004","url":null,"abstract":"Venous thromboembolism (VTE), including deep vein thrombosis (DVT) and pulmonary embolism (PE), is a major health concern and cause of mortality worldwide. Among the well-established risk factors for VTE, congestive heart failure (CHF) is a well-known major risk factor, especially in hospitalized patients. In CHF patients, the risk of VTE is increased because of multi-factorial causes including blood stasis due to decreased ejection fraction (EF), immobilization, endothelial dysfunction, and hemostatic abnormalities. Thus, VTE is approximately three times more likely to develop in CHF patients than in individuals without CHF. Most previous studies regarding VTE in CHF patients were conducted in Caucasian populations. The reported incidence of VTE in CHF patients has widely ranged from 1.63% to 59%. Recently, several epidemiologic studies in the Asian population demonstrated that VTE incidence is rapidly increasing, making VTE an important health concern in Asian countries. In this respect, we hypothesized that CHF is an important risk factor for VTE in Asian populations. To date, however, despite the clinical significance of CHF as a likely VTE risk factor, its contribution to VTE risk has not been established in an Asian population. Therefore, we conducted this nationwide epidemiologic study using the Korean Health Insurance Review and Assessment Service (HIRA) database to analyze the VTE prevalence in CHF patients. We also performed a retrospective observation study to investigate potential VTE risks in Korean CHF patients. Methods","PeriodicalId":249962,"journal":{"name":"Clinical & Experimental Thrombosis and Hemostasis","volume":"60 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130453041","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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