静脉注射免疫球蛋白治疗获得性血管性假血友病综合征

获得性血管性假血友病与许多疾病(包括自身免疫失调)有关.在一些患者体内发现有血管性假血友病因子(vWF)的抑制剂,而大多数vWF缺乏症的原因不明.作者曾报道用静脉注射免疫球蛋白(IVIG)治疗1例伴有与vWF抑制剂有关的狼疮样抗凝因子之患者,至少在2～3周内纠正了其vWF缺乏症及ⅡA型多聚体型,使其在髋关节置换术中未发生出血.     

血清淀粉样蛋白A血管性血友病因子降钙素原及可溶性程序性死亡受体-1在重症监护室脓毒症患者预后和细菌类型的诊断价值

目的 探讨血清淀粉样蛋白A(SAA)、血管性血友病因子(vWF)、降钙素原(PCT)及可溶性程序性死亡受体-1(sPD-1)在重症监护室(ICU)脓毒症患者中的水平及预测患者预后、细菌类型中的价值.方法 选取2020年1月至2021年5月在平顺县人民医院ICU治疗的脓毒症患者132例,分析不同病原菌、预后患者SAA、v...     

vWF/ADAMTS13在慢性肾脏病中的临床意义

目的探讨血管性血友病因子(vWF)及其裂解蛋白酶(ADAMTS13)在慢性肾脏病中的临床意义。方法选取慢性肾脏病患者47例为实验组(A组),其中原发性肾小球疾病(A1组)24例,狼疮性肾炎(A2组)23例;另设体检健康成人30例为正常对照组(B组)。A1组患者均行肾活检,其中非膜性肾病15例(C1组),膜性肾病9例(C2组)。ELISA法、FRETS-vWF73法分别检测血浆中vWF含量、ADAMTS13活性,免疫组化法检测vWF和ADAMTS13在肾脏的表达。结果与B组相比,A1、A2组血浆中ADAMTS13活性降低,而vWF浓度升高(P<0.01);A组患者血浆vWF/ADAMTS13与24-h尿蛋白定量呈正相关(r=0.477,P<0.01);C2组ADAMTS13的表达明显低于C1组(P<0.05)。结论血浆中vWF与ADAMTS13的平衡失调可能与慢性肾脏病的临床和病理类型有关。     

贝那普利对高血压病患者血浆同型半胱氨酸水平和血管内皮功能的影响

目的:研究贝那普利对原发性高血压患者血浆同型半胱氨酸(Hcy)水平和血管内皮功能的影响。方法:77例受试者随机分为治疗组和对照组,对照组给予常规降压治疗,治疗组在此基础上加用贝那普利,观察并比较治疗前、后血浆Hcy水平、内皮依赖性血管舒张功能(FMD)、一氧化氮(NO)浓度及血管性假性血友病因子(v WF)水平等指标的变化。结果:治疗组治疗后与治疗前及对照组治疗后比较Hcy及v WF水平均降低,NO浓度升高,FMD明显升高(P<0·001或P<0·05)。结论:贝那普利降压治疗同时可降低血浆Hcy水平,改善高血压患者的血管内皮功能。     

血浆因子Ⅷ相关抗原在临床上检测的价值

血浆因子Ⅷ是一种高分子量糖蛋白,包括三种主要生物学活性:因子Ⅷ凝血活性(简称ⅧC);因子Ⅷ相关抗原(简称ⅧR:Ag);因子Ⅷ相关的血管性假血友病因子(简称ⅧR:WF)。研究因子Ⅷ相关活性及其相关抗原的定位,在临床上不仅对有些遗传性出血性疾病(血友病、血管性假血友病)的诊断、鉴别诊断、分型等具有重要意义,而且为研究各     

紫癜性肾炎患者血浆TM、vWF和IL-8水平的变化

目的探讨紫癜性肾炎患儿血浆血栓调节蛋白(TM)、血管性假性血友病因子(vWF)和白细胞介素8(IL-8)水平的变化及其与紫癜性肾炎临床分型的相关性。方法选择过敏性紫癜患儿65例(HSP组);其中过敏性紫癜性肾炎(HSPN)51例,无肾损害者14例(HSP单纯型)。51例HSPN患儿分为:孤立性血尿或孤立性蛋白尿18例(HSPN 1型),血尿和蛋白尿14例(HSPN 2型),急性肾炎型11例(HSPN 3型),肾病综合征型8例(HSPN 4型)。对照组为我科门诊体检的健康儿童19例。分别测定血浆TM、vWF、IL-8水平,分析血浆TM、vWF、IL-8与紫癜性肾炎临床分型的关系。结果 HSP组血浆TM、vWF、IL-8高于对照组(P<0.05),HSPN 2、3、4型患儿血浆TM、vWF、IL-8明显高于单纯型(P<0.05)。结论紫癜性肾炎患儿血浆TM、vWF、IL-8水平随着临床分型不同而变化。联合测定有助于紫癜性肾炎的早期诊断和治疗。     

血友病的诊断和治疗

广义的血友病(hemophilia)是指一组因遗传性凝血活酶生成障碍引起的出血性疾病.包括血友病A、血友病B、遗传性FⅪ缺乏症(过去称为血友病丙)及血管性血友病(vWD).……     

肺癌患者凝血功能与肺癌临床分期的关系

目的探讨肺癌患者凝血功能指标与肺癌临床分期的关系。方法对我院确诊的100例肺癌患者和20例正常人的凝血功能指标(包括抗凝血酶Ⅲ、D-二聚体、血浆纤维蛋白原、血管性血友病因子)用免疫比浊法或ELISA进行检测,并分析结果与肺癌临床分期的关系。结果肿瘤患者抗凝血酶Ⅲ、D-二聚体、血浆纤维蛋白原、血管性血友病因子的水平都要比正常人高;Ⅲ期、Ⅳ期肺癌患者的抗凝血酶Ⅲ、D-二聚体、血浆纤维蛋白原、血管性血友病因子的水平要比Ⅰ期肺癌患者明显增高(P<0.05),差异有统计学意义;Ⅱ期与Ⅰ期相比增高不明显(P>0.05),差异没有统计学意义。结论肺癌患者凝血功能指标异常,随着临床分期的增高凝血系统功能紊乱也在不断的加重。     

The changes of plasma thrombomodulin, von Willebrand factor and interleukin 8 in children with purpura nephritis

目的 探讨紫癜性肾炎患儿血浆血栓调节蛋白(TM)、血管性假性血友病因子(vWF)和白细胞介素8(IL-8)水平的变化及其与紫癜性肾炎临床分型的相关性.方法 选择过敏性紫癜患儿65例(HSP组);其中过敏性紫癜性肾炎(HSPN) 51例,无肾损害者14例(HSP单纯型).51例HSPN患儿分为:孤立性血尿或孤立性蛋白尿18例(HSPN 1型),血尿和蛋白尿14例(HSPN 2型),急性肾炎型11例(HSPN 3型),肾病综合征型8例(HSPN 4型).对照组为我科门诊体检的健康儿童19例.分别测定血浆TM、vWF、IL-8水平,分析血浆TM、vWF、IL-8与紫癜性肾炎临床分型的关系.结果 HSP组血浆TM、vWF、Ⅱ-8高于对照组(P＜0.05),HSPN 2、3、4型患儿血浆TM、vWF、IL-8明显高于单纯型(P＜0.05).结论 紫癜性肾炎患儿血浆TM、vWF、IL-8水平随着临床分型不同而变化.联合测定有助于紫癜性肾炎的早期诊断和治疗.     

血管性血友病因子层析纯化方法研究进展

从血浆中分离纯化出各种有价值的蛋白是生物技术领域的重要产业。血管性血友病因子可从血浆中提取,能有针对性地治疗遗传性和获得性血管性血友病,为患者提供最佳的治疗手段。蛋白层析纯化法能得到纯度高,活性佳的蛋白分子,是血管性血友病因子提纯的主要方法。现综述离子交换法、亲和层析法和凝胶过滤法3种重要的纯化方法在血管性血友病因子制备中的应用。     

Von Willebrand disease: an overview

Most commonly inherited bleeding disorder, first described in Aland Islands by Erik von Willebrand. It occurs as a result of decrease in plasma levels or defect in von Willebrand factor which is a large multimeric glycoprotein. Monomers of this glycoprotein undergo N-glycosylation to form dimers which get arranged to give multimers. Binding with plasma proteins (especially factor VIII) is the main function of von Willebrand factor. The disease is of two forms: Inherited and acquired forms. Inherited forms are of three major types. They are type 1, type 2, and type 3; in which type 2 is sub-divided into 2A, 2B, 2M, 2N. Type 1 is more prevalent than all other types. Mucocutaneous bleeding is mild in type 1 whereas it is mild to moderate in types 2A, 2B, and 2M. Type 2N has similar symptoms of haemophilia. The pathophysiology of each type depends on the qualitative or quantitative defects in von Willebrand factor. The diagnosis is based on von Willebrand factor antigen, von Willebrand factor activity assay, FVIII coagulant activity and some other additional tests. Results should be analyzed within the context of blood group. von Willebrand factor multimer analysis is essential for typing and sub typing the disease. The management of the disease involves replacement therapy, non-replacement therapy and other therapies that include antifibrinolytics and topical agents.     

Human plasma von Willebrand factor/factor VIII complex (Haemate P/Humate-P): in von Willebrand disease and haemophilia A

Haemate P/Humate-P is a pasteurised human plasma-derived concentrate containing coagulation factor VIII and a near-normal spectrum of von Willebrand factor multimers, including high-molecular weight multimers, for intravenous use in patients with von Willebrand disease or haemophilia A. Extensive clinical experience over the past 25 years has shown that Haemate P/Humate-P provides effective haemostatic control for the prevention and treatment of bleeds in patients with these conditions, with no confirmed cases of viral or prion transmission occurring during this time. In small prospective and retrospective noncomparative studies, Haemate P/Humate-P provided effective haemostatic control for the prevention and treatment of bleeding episodes in the vast majority of paediatric and adult patients with von Willebrand disease. Haemate P/Humate-P was generally well tolerated in patients with von Willebrand disease or haemophilia A.     

血管性血友病致反复失血性休克1例并文献复习

血管性血友病是常见的遗传性出血性疾病,临床表现多样化,其诊断及治疗仍面临挑战.本文报道血管性血友病致反复失血性休克1例,并进行相关文献复习,为深入认识血管性血友病,及时进行临床诊断及治疗提供参考.     

Short- and long-term effects of therapy with interferon-alpha and pegylated interferon-alpha/ribavirin on platelet plug formation and von Willebrand factor release in patients with chronic hepatitis C

BACKGROUND: A pegylated interferon-alpha-induced decrease in platelet counts may become a limiting factor for continuation of therapy. AIM: To evaluate the effect of pegylated interferon-alpha administration on platelet plug formation and von Willebrand factor antigen release in patients with chronic hepatitis C. METHODS: Thirty patients with chronic hepatitis C (genotype 1; fibrosis 1-3: n = 16, cirrhosis: n = 14) received a single dose of 9 MU interferon-alpha2a, followed by weekly administration of 180 mug of pegylated interferon-alpha2a/ribavirin for 48 weeks. Platelet counts, platelet function (collagen-epinephrine-induced closure time) and von Willebrand factor antigen were measured. RESULTS: Platelet counts and collagen-epinephrine-induced closure time decreased by 13% and 16%, respectively, 24 h after the first dose of interferon-alpha2a, and von Willebrand factor antigen levels increased by 31% (P < 0.01) compared with baseline. During a 48-week observation period, platelet counts decreased by a maximum of 33% (P < 0.001), von Willebrand factor antigen levels increased by 69% (P < 0.001) whereas collagen-epinephrine-induced closure time did not change. In noncirrhotic patients, the increase of von Willebrand factor antigen levels was maintained throughout therapy without a change in collagen-epinephrine-induced closure time. In contrast, in cirrhotics, von Willebrand factor antigen levels did not increase, while collagen-epinephrine-induced closure time was prolonged. CONCLUSION: Single-dose interferon-alpha decreases platelet counts but improves platelet function, possibly by the release of von Willebrand factor antigen. Accordingly, long-term antiviral treatment had no effect on collagen-epinephrine-induced closure time, despite the decrease in platelet count in noncirrhotic patients. Such a compensation of decreased platelet counts by increased von Willebrand factor antigen level did not occur in cirrhotics.     

Therapeutic approaches to acquired von Willebrand syndrome

Acquired von Willebrand syndrome (AVWS) is a rare acquired bleeding disorder similar to the congenital von Willebrand disease (VWD) in terms of laboratory findings. Diagnosis of AVWS can be very difficult, with treatment normally taking an empirical form. Although more than 200 cases have been reported since 1968, no retrospective or prospective studies are available on AVWS. Recently, an International Registry on AVWS, gathering data directly from worldwide Departments of Haematology-Oncology and Haemophilia Centres, has been organised by a group working on behalf of the Subcommittee on VWF in the Scientific Standardisation Committee (SSC) of International Society on Thrombosis and Haemostasis (ISTH). Information about an additional 211 AVWS patients is now available, with more detailed data on demography, type of haemorrhage, diagnostic tests for AVWS and management of bleeding episodes. The additional 211 AVWS cases are associated with lymphoproliferative (47%) or myeloproliferative (19%) disorders, cardiovascular diseases, neoplasia (7%) and other miscellaneous diseases (14%). Bleeding episodes of AVWS patients were managed by different compounds including desmopressin (22%), FVIII/VWF concentrates (26%) and high-dose immunoglobulin (10%), plasmapheresis (2%), steroids (5%) and immunosuppressive drugs (20%). Based on complied data, we can conclude that none of the therapeutic approaches proposed are 100% effective in all AVWS cases. Therefore, treatment must be customized for each patient according to the underlying disorder, as well as to the type and the severity of bleeding episode and must be targeted to each specific case.     

Therapeutic approaches to acquired von Willebrand syndrome

Acquired von Willebrand syndrome (AVWS) is a rare acquired bleeding disorder similar to the congenital von Willebrand disease (VWD) in terms of laboratory findings. Diagnosis of AVWS can be very difficult, with treatment normally taking an empirical form. Although more than 200 cases have been reported since 1968, no retrospective or prospective studies are available on AVWS. Recently, an International Registry on AVWS, gathering data directly from worldwide Departments of Haematology-Oncology and Haemophilia Centres, has been organised by a group working on behalf of the Subcommittee on VWF in the Scientific Standardisation Committee (SSC) of International Society on Thrombosis and Haemostasis (ISTH). Information about an additional 211 AVWS patients is now available, with more detailed data on demography, type of haemorrhage, diagnostic tests for AVWS and management of bleeding episodes. The additional 211 AVWS cases are associated with lymphoproliferative (47%) or myeloproliferative (19%) disorders, cardiovascular diseases, neoplasia (7%) and other miscellaneous diseases (14%). Bleeding episodes of AVWS patients were managed by differnent compounds including desmopressin (22%), FVIII/VWF concentrates (26%) and high-dose immunoglobulin (10%), plasmapheresis (2%), steroids (5%) and immunosuppressive drugs (20%). Based on complied data, we can conclude that none of the therapeutic approaches proposed are 100% effective in all AVWS cases. Therefore, treatment must be customised for each patient according to the underlying disorder, as well as to the type and the severity of bleeding episode and must be targeted to each specific case.     

血管性血友病因子对冠心病发生影响的研究进展

von Willebrand因子又称血管性血友病因子,通过特异的血小板膜受体(糖蛋白Ιb-IX复合物)介导血小板与内皮下层的黏附,是一个反应内皮损伤的标志物,其增高有利于血小板的黏附。vWF与冠心病的发生相关,是反映内皮细胞受损的可靠指标之一。本研究通过对vWF生物学特点、生理功能、作用机理及对冠心病发生影响的相关论文的研究整理,为临床探讨冠心病影响因素及治疗提供参考依据。     

The safety of plasma-derived von Willebrand/factor VIII concentrates in the management of inherited von Willebrand disease

Until the mid-80s, cryoprecipitate has been the mainstay of treatment of patients with von Willebrand disease who were unresponsive to desmopressin. The advent of virally-inactivated factor VIII (FVIII) concentrates containing von Willebrand factor (VWF), originally devoted to hemophiliacs, provided a better therapeutic approach to von Willebrand disease. These VWF/FVIII concentrates were introduced in clinical practice after the positive results obtained in several prospective and retrospective clinical studies. They are safe and can be suitable also for home treatment. Allergic or anaphylactic reactions are limited to the rare patients with deletions of VWF gene. In repeated infusions during surgery, the dosage and timing of administration should be planned to keep FVIII below 150 – 200 U/dl to avoid any possible risk of thrombosis.     

Structure and function of snake venom proteins affecting platelet plug formation

Many snake venom proteins have been isolated that affect platelet plug formation by interacting either with platelet integrins, membrane glycoprotein Ib (GPIb), or plasma von Willebrand factor (VWF). Among them, disintegrins purified from various snake venoms are strong inhibitors of platelet aggregation. Botrocetin and bitiscetin derived from Bothrops jararaca and Bitis arietans venom, respectively, induce VWF-dependent platelet agglutination in vitro. Several GPIb-binding proteins have also been isolated from snake venoms. In this review, we focus on the structure and function of those snake venom proteins that influence platelet plug formation. These proteins are potentially useful as reagents for the sub-diagnosis of platelet disorder or von Willebrand disease, as well as for clinical and basic research of thrombosis and hemostasis.     

Drug therapy reviews: clinical use of hemostatic agents.

Systemic hemostatic agents are reviewed. Among the agents discussed are vitamin K preparations (phytonadione, menadione, menadione sodium bisulfite, menadiol sodium diphosphate); and blood products (whole blood, plasma, cryoprecipitate, factor VIII concentrates, factor IX concentrates and fibrinogen concentrates). Normal and abnormal hemostasis and fibrinolysis are discussed, as is the general management of systemic hemostatic defects. Specific disorders covered are clotting factor deficiencies, hemophilia A, factor VIII inhibitors, von Willebrand disease, hemophilia B (Christmas disease), other congenital coagulation disorders, acquired deficiency of factors II, VII, IX and X, and defibrination syndrome.