A prospective, randomized trial of prednisone and cyclophosphamide in the treatment of patients with factor VIII autoantibodies.

Abstract:

:Thirty-one non-hemophilic patients with acquired antibodies to factor VIII were entered into a prospective, randomized, multi-institutional trial to determine the safety and efficacy of prednisone (P), cyclophosphamide (C), or the combination in the treatment of this disorder. Patients were eligible if they had antibody demonstrated by the Bethesda assay, had not received these agents previously, and gave informed consent. All patients were treated initially with P, 1 mg/kg, for 3 weeks. If the antibody persisted and there was no rise in factor VIII activity, patients were randomized to either continue P for an additional 6 weeks; taper P and begin C, 2 mg per kg; or continue P and add C. Antibody disappeared in 10 during the initial P therapy, and in three of four others randomized to continue on P; one patient was discontinued because of an herpetic infection. Antibody disappeared in three of six patients treated with C alone, and five of ten given C and P. The titer of antibody was significantly lower in responders than in non-responders (p = 0.003), but seven patients with titers of more than five Bethesda units had complete remissions. There was no difference in antibody titers between those responding to P and those responding to C. We conclude that all patients with acquired antibodies to factor VIII should receive initial management with P, and that C is effective as second-line therapy for many of those who are steroid-resistant.

journal_name

Thromb Haemost

authors

Green D,Rademaker AW,Briët E

subject

Has Abstract

pub_date

1993-11-15 00:00:00

pages

753-7

issue

5

eissn

0340-6245

issn

2567-689X

journal_volume

70

pub_type

临床试验,杂志文章,多中心研究,随机对照试验
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    abstract::Platelets are not only centrally involved in haemostasis, but also in antimicrobial defence and inflammation. Since evaluation of platelet physiology in the particular patient group of preterm and term neonatal infants is highly restricted for ethical reasons, there are hardly any data available in healthy and much le...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,评审

    doi:10.1160/TH14-05-0476

    authors: Andres O,Schulze H,Speer CP

    更新日期:2015-01-01 00:00:00

  • Dysfunctional plasminogen in full term newborn--study of active site of plasmin.

    abstract::The functional activity and active site of plasmin in full-term newborns have been studied and compared to those in adults in order to investigate the nature of the abnormality found in newborn plasminogen described in a previous paper. The functional activity of newborn plasminogen measured on chromogenic substrate w...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Benavent A,Estellés A,Aznar J,Martinez-Sales V,Gilabert J,Fornas E

    更新日期:1984-02-28 00:00:00

  • Home-diagnosis of deep venous thrombosis with impedance plethysmography.

    abstract::In order to assess the value of I.P.G. for the diagnosis of D.V.T. in general practice, an I.P.G. was carried out by a skilled technician in 255 consecutive patients with suspected D.V.T. at home. Ascending venography was carried out in 185 of these patients. In addition, blood for assay of AT III, platelet count, fib...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Peters SH,Jonker JJ,de Boer AC,den Ottolander GJ

    更新日期:1982-12-27 00:00:00

  • Interaction of bovine platelets with bovine glomerular basement membrane.

    abstract::The interaction of bovine platelets with bovine glomerular basement membrane has been studied by aggregometry, transmission and scanning electron microscopy and measurement of [3H] serotonin release. In the absence of added calcium platelets adhere to basement membrane but fail to undergo the release reaction or aggre...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Davis JL,Chandler DK,Riquetti P,Rogers S,Kocan KM,Ownby CL,Hudson BG,Carraway KL

    更新日期:1979-12-21 00:00:00

  • The mild phenotype in severe hemophilia A with Arg1781His mutation is associated with enhanced binding affinity of factor VIII for factor X.

    abstract::The clinical severity in some patients with haemophilia A appears to be unrelated to the levels of factor (F)VIII activity (FVIII:C), but mechanisms are poorly understood. We have investigated a patient with a FVIII gene mutation at Arg1781 to His (R1781H) presenting with a mild phenotype despite FVIII:C of 0.9 IU/dl....

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1160/TH12-10-0762

    authors: Yada K,Nogami K,Wakabayashi H,Fay PJ,Shima M

    更新日期:2013-06-01 00:00:00

  • The platelet adhesion capacity to subendothelial matrix and collagen in a flow model during storage of platelet concentrates for 7 days.

    abstract::The influence of storage of platelet concentrates (PC) on the adhesion capacity of platelets was studied. Twenty-four PC, 12 prepared by the buffy coat (BC) method and 12 by the platelet-rich plasma (PRP) method, were stored for 7 days at room temperature. On days 1, 3 and 7 of storage, the platelet adhesion capacity ...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Boomgaard MN,Gouwerok CW,Homburg CH,de Groot G,IJsseldijk MJ,de Korte D

    更新日期:1994-10-01 00:00:00

  • Pathophysiological relevance of macrophage subsets in atherogenesis.

    abstract::Macrophages are highly heterogeneous and plastic cells. They were shown to play a critical role in all stages of atherogenesis, from the initiation to the necrotic core formation and plaque rupture. Lesional macrophages primarily derive from blood monocyte, but local macrophage proliferation as well as differentiation...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,评审

    doi:10.1160/TH16-08-0593

    authors: Liberale L,Dallegri F,Montecucco F,Carbone F

    更新日期:2017-01-05 00:00:00

  • Low Affinity Platelet Factor 4 and High Affinity Platelet Factor 4 - Two Antiheparin Factors Secreted by Human Platelets.

    abstract:: ...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0038-1657075

    authors: Niewiarowski S,Rucinski B,Budzynski AZ

    更新日期:1979-07-01 00:00:00

  • Genetic diagnosis of factor V Leiden using heteroduplex technology.

    abstract::A new genetic test has been developed for detection of the mutation known as factor V Leiden. The test employs heteroduplex technology and comprises a single PCR reaction followed immediately by PCR product analysis. It therefore represents the minimum practical route from blood/tissue sample to genetic result. A coho...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Bowen DJ,Standen GR,Granville S,Bowley S,Wood NA,Bidwell J

    更新日期:1997-01-01 00:00:00

  • Sympathoadrenal activation and muscarinic receptor stimulation induce acute release of tissue-type plasminogen activator but not von Willebrand factor across the human forearm.

    abstract::We have previously shown that both mental stress and administration of the muscarinic receptor agonist methacholine induce an acute release of tissue-type plasminogen activator (t-PA) across the human forearm. There are data indicating that the regulated acute release of t-PA from the endothelium is closely interrelat...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Jern C,Selin L,Tengborn L,Jern S

    更新日期:1997-08-01 00:00:00

  • A slow clearing, fibrin-specific, PAI-1 resistant variant of t-PA (T103N, KHRR 296-299 AAAA).

    abstract::Site directed mutagenesis was used to construct a t-PA variant that contains an additional glycosylation site in the first kringle domain (T103N) combined with a tetra-alanine substitution in the protease domain (KHRR 296-299 AAAA). This combination variant has a plasma clearance rate that is 4.5-fold slower in rats a...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Paoni NF,Keyt BA,Refino CJ,Chow AM,Nguyen HV,Berleau LT,Badillo J,Peña LC,Brady K,Wurm FM

    更新日期:1993-08-02 00:00:00

  • A randomised, double blind comparison of tecarfarin, a novel vitamin K antagonist, with warfarin. The EmbraceAC Trial.

    abstract::Tecarfarin is a novel vitamin K antagonist that is metabolised by carboxyl estererase, thereby eliminating the variability associated with cytochrome-mediated metabolism. EmbraceAC was designed to compare the quality of anticoagulation with tecarfarin and warfarin as determined by time in therapeutic range (TTR). In t...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1160/TH15-11-0910

    authors: Whitlock RP,Fordyce CB,Midei MG,Ellis D,Garcia D,Weitz JI,Canafax DM,Albrecht D,Milner PG

    更新日期:2016-08-01 00:00:00

  • Bernard-Soulier syndrome with severe bleeding: absent platelet glycoprotein Ib alpha due to a homozygous one-base deletion.

    abstract::Bernard-Soulier syndrome is a rare congenital platelet disorder that affects a surface membrane adhesion receptor, glycoprotein (GP) Ib-V-IX. Both the genetic defects and the bleeding diatheses associated with the syndrome are heterogeneous due, in part, to the complexity of the involved receptor which consists of fou...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Li C,Pasquale DN,Roth GJ

    更新日期:1996-11-01 00:00:00

  • Monocytes of Different Subsets in Complexes with Platelets in Patients with Myocardial Infarction.

    abstract::Acute myocardial infarction (AMI) is associated with activation of various cells, including platelets that form monocyte-platelet complexes (MPCs). Here, we analysed MPC in vivo and in vitro and investigated the abilities of different monocyte subclasses to form MPC, the characteristics of the cells involved in MPC fo...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1055/s-0038-1673342

    authors: Loguinova M,Pinegina N,Kogan V,Vagida M,Arakelyan A,Shpektor A,Margolis L,Vasilieva E

    更新日期:2018-11-01 00:00:00

  • A novel mechanism of transcriptional repression of p27kip1 through Notch/HRT2 signaling in vascular smooth muscle cells.

    abstract::Vascular smooth muscle cell (VSMC) proliferation occurs in vascular obstructive events such as atherosclerosis and restenosis. We previously showed that Notch receptors are induced in smooth muscle cells during vascular remodeling. Our goal was to determine the mechanisms employed by Notch signaling to regulate prolif...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1160/TH06-04-0224

    authors: Havrda MC,Johnson MJ,O'Neill CF,Liaw L

    更新日期:2006-09-01 00:00:00

  • Low density lipoprotein receptor-related protein: regulation of the plasma membrane proteome.

    abstract::Proteins in the plasma membrane anchor the cell within its microenvironment and sense changes occurring outside the cell. The anchoring interactions are cell type-specific and may involve adjacent cells or extracellular matrix proteins (ECMPs). In development, wound healing, and in various forms of pathology, includin...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,评审

    doi:10.1160/TH04-01-0023

    authors: Gonias SL,Wu L,Salicioni AM

    更新日期:2004-06-01 00:00:00

  • Remodeling of carotid arteries is associated with increased expression of thrombomodulin in a mouse transverse aortic constriction model.

    abstract::Thrombomodulin (TM) is an endothelial glycoprotein that functions as a thrombin cofactor in the activation of protein C. Recent evidence has revealed that TM has unique effects on cellular proliferation, adhesion, and inflammation. We examined TM expression in the arterial remodeling process with different shear condi...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Li YH,Hsieh CY,Wang DL,Chung HC,Liu SL,Chao TH,Shi GY,Wu HL

    更新日期:2007-04-01 00:00:00

  • Intra-coronary thrombolysis with streptokinase or lys-plasminogen/urokinase in acute myocardial infarction: effects on recanalization and blood fibrinolysis.

    abstract::Forty-two patients with total occlusion of a coronary vessel were treated with intracoronary fibrinolytic agents. Four therapeutic protocols were compared: group I received streptokinase (SK) as a continuous infusion; group II and III received SK as a bolus at different doses and group IV received lysplasminogen (Pg) ...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: de Prost D,Guerot C,Laffay N,Horellou MH,Samama M

    更新日期:1983-12-30 00:00:00

  • Molecular mechanism and classification of von Willebrand disease.

    abstract::The characterization of mutations in von Willebrand disease provides useful insight into the synthesis, structure, and function of von Willebrand factor. This growing body of information has prompted a reclassification of vWD types that is intended to reflect distinct pathophysiologic mechanisms. Despite this apparent...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,评审

    doi:

    authors: Sadler JE,Matsushita T,Dong Z,Tuley EA,Westfield LA

    更新日期:1995-07-01 00:00:00

  • Factor VII antigen levels in a healthy blood donor population.

    abstract::We determined factor VII antigen (FVIIag) levels in 705 healthy blood donors ranging in age from 17 to 79 years using a two-site solid-phase enzyme immunoassay developed in our laboratory. The mean (+/- SD) FVIIag level for the total population was 102 +/- 31%. FVIIag levels for men (n = 375) and women (n = 330) were ...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Howard PR,Bovill EG,Pike J,Church WR,Tracy RP

    更新日期:1994-07-01 00:00:00

  • Dysfibrinogenemia (fibrinogen Dusard) associated with impaired fibrin-enhanced plasminogen activation.

    abstract::The fibrin-mediated enhancement of the activation of plasminogen by tissue-type plasminogen activator observed with normal fibrin, is strongly decreased with fibrin Dusard, although the binding of tissue-type plasminogen activator to this fibrin is normal. This impaired fibrin-mediated plasminogen activation is most l...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Lijnen HR,Soria J,Soria C,Collen D,Caen JP

    更新日期:1984-02-28 00:00:00

  • Aggregation of human platelets by acidic mucopolysaccharide extracted from Stichopus japonicus Selenka.

    abstract::The acidic mucopolysaccharide extracted from sea cucumber (Stichopus japonicus Selenka) (SJAMP) has been shown to cause platelets to aggregate. Using citrated platelet-rich plasma (PRP), washed platelets and formaldehyde-fixed platelets from humans, we investigated the effects of platelet inhibitors and various plasma...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Li JZ,Lian EC

    更新日期:1988-06-16 00:00:00

  • Deep vein thrombosis in obstetric patients: diagnosis and risk factors.

    abstract::This study was performed to assess the prevalence of deep vein thrombosis (DVT) in consecutive obstetric patients with clinical symptoms of DVT, using impedance plethysmography (IPG) as the diagnostic method and to establish the safety of withholding anticoagulant therapy in patients with a repeatedly normal IPG. In a...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: de Boer K,Büller HR,ten Cate JW,Levi M

    更新日期:1992-01-23 00:00:00

  • Three abnormal fibrinogen variants with the same amino acid substitution (gamma 275 Arg----His): fibrinogens Bergamo II, Essen and Perugia.

    abstract::We report on three unrelated individuals with the same uncommon type of dysfibrinogenemia, originating from Bergamo, Essen and Perugia. None of them showed bleeding symptoms while the Bergamo patient and members of her family presented with a thrombotic tendency. The presence of a defective fibrinogen was suggested by...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Reber P,Furlan M,Henschen A,Kaudewitz H,Barbui T,Hilgard P,Nenci GG,Berrettini M,Beck EA

    更新日期:1986-12-15 00:00:00

  • Endothelial cell protein S synthesis is upregulated by the complex of IL-6 and soluble IL-6 receptor.

    abstract::We have recently demonstrated that the proinflammatory cytokine, interleukin-6 (IL-6), could upregulate the production of protein S in the human hepatoma cell line, HepG-2, but not in endothelial cells. In this study, we have demonstrated that the combination of exogenous IL-6 and soluble IL-6 receptor (sIL-6R) could ...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Hooper WC,Phillips DJ,Evatt BL

    更新日期:1997-05-01 00:00:00

  • Antithrombotic therapy of acute stroke: thrombolytic agents.

    abstract::Following the initial report of the clinical use of plasminogen activators (PAs) in cerebrovascular thrombosis in 1958, interest in the efficacy of this approach accelerated only when the need for acute intervention in stroke was recognized. The use of PAs in acute ischemic stroke is based on the observation that appr...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,评审

    doi:

    authors: del Zoppo GJ

    更新日期:1997-07-01 00:00:00

  • Treatment with dabigatran or warfarin in patients with venous thromboembolism and cancer.

    abstract::The efficacy and safety of dabigatran for treatment of venous thromboembolism (VTE) were demonstrated in two trials. It is unclear if the results pertain to patients with cancer and VTE. Data from two randomised trials comparing dabigatran and warfarin for acute VTE were pooled. Primary efficacy outcome was symptomati...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:10.1160/TH14-11-0977

    authors: Schulman S,Goldhaber SZ,Kearon C,Kakkar AK,Schellong S,Eriksson H,Hantel S,Feuring M,Kreuzer J

    更新日期:2015-07-01 00:00:00

  • Induction of vasoactive substances differs in LPS-induced and TF-induced DIC models in rats.

    abstract::We have investigated the role of two vasoactive substances, nitric oxide (NO)and endothelin (ET), in the pathophysiology of disseminated intravascular coagulation (DIC), using two types of DIC models. Experimental DIC was induced by sustained infusion of 0.1, 1, 10, or 50 mg/kg lipopolysaccharide (LPS), or 3.75 U/kg t...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Asakura H,Okudaira M,Yoshida T,Ontachi Y,Yamazaki M,Morishita E,Miyamoto K,Nakao S

    更新日期:2002-10-01 00:00:00

  • Effects of tris on responses of human and rabbit platelets to aggregating agents.

    abstract::Despite reports that Tris [tris (hydroxymethyl)aminomethane] affects platelets, it is often used to buffer suspending media. Human or rabbit platelets were washed and resuspended in Tyrode solution containing apyrase and 0.35% albumin. Addition of 15 mM Tris partially inhibited primary aggregation induced by 10 microM...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章

    doi:

    authors: Packham MA,Guccione MA,Nina M,Kinlough-Rathbone RL,Mustard JF

    更新日期:1984-04-30 00:00:00

  • Extracellular regulation of TGF-beta activity in wound repair: growth factor latency as a sensor mechanism for injury.

    abstract::The transforming growth factor-beta (TGFbeta) family of growth factors are major regulators of wound repair, scar formation, and fibrosis. One of the prominent features of TGFbeta biology is the fact that this growth factor is secreted as a latent precursor, which may be directed to and stored at specific sites in the...

    journal_title:Thrombosis and haemostasis

    pub_type: 杂志文章,评审

    doi:10.1160/TH04-05-0324

    authors: Brunner G,Blakytny R

    更新日期:2004-08-01 00:00:00