Abstract：
:Less than 60 cases of acquired factor (F)XIII deficiencies have been reported, most having distinct clinical features. To illustrate the therapeutic challenges of acquired FXIII inhibitors, we report a case of a 65-year-old patient with no previous bleeding history who suddenly developed massive haemorrhages associated to a strong and isolated FXIII inhibitor. No underlying disorder has been detected till now after three years of follow-up. Despite aggressive treatment with prednisone, rituximab, cyclophosphamide, immunoglobulin, immunoadsorption and immune tolerance his inhibitor is still present, although at low titre and with a clinical benefit since the patient has no more bleed since more than one year. Moreover the patient had a venous thromboembolic complication. After a review of the management of acquired FXIII deficiency patients and based on the management of acquired haemophilia we discuss a possible strategy for such difficult cases.
journal_name
Thromb Haemostjournal_title
Thrombosis and haemostasisauthors
Boehlen F,Casini A,Chizzolini C,Mansouri B,Kohler HP,Schroeder V,Reber G,de Moerloose Pdoi
10.1160/TH12-08-0604subject
Has Abstractpub_date
2013-03-01 00:00:00pages
479-87issue
3eissn
0340-6245issn
2567-689Xpii
12-08-0604journal_volume
109pub_type
杂志文章,评审abstract：:The fibrinolytic capacity of 121 patients with a history of venous thrombosis and/or pulmonary embolism was studied by venous occlusion technique, at earliest 3 months after the last thromboembolic episode. After discontinuation of oral anticoagulation treatment the clinical course of the patients was followed and new...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1984-10-31 00:00:00
abstract：:To assess interobserver variability of venography for screening for postoperative deep venous thrombosis (DVT), we used 185 bilateral ascending contrast venograms (366 lower limbs) which were performed using the long-leg film technique, in the frame of a trial of the efficacy of two low-molecular-weight-heparin fracti...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章
doi：
更新日期：1993-10-18 00:00:00
abstract：:We report here on a patient whose abnormal fibrin clot was detected via the measurement of clot rigidity with a simple buoyant inner cylinder elastometer. The patient's clinical coagulation studies were all within normal limits except for prolonged thrombin and reptilase clotting times and high level of fibrin split p...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1979-10-31 00:00:00
abstract：:Hot water extracts of Mo-er (1 gm by 15 ml of water), an oriental food (Auricularia auricula), inhibit strongly both human and rat platelet ADP-induced aggregation. HPLC analysis of two varieties of Mo-er, A. auricula and A. polytricha (a black tree fungus), shows that they contain adenosine (Ado), 133 and 154 microgr...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1982-10-29 00:00:00
abstract：:ADP or thrombin caused a transient change in creatine phosphate (CP) content of rat platelets. At 25 degrees C the CP content decreased most 30 sec after the addition of ADP or thrombin and recovered thereafter. ATP or AMP was without effect. The rat platelets aggregated at 25 degrees C by ADP or thrombin, but the cha...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1979-08-31 00:00:00
abstract：:Today, direct oral anticoagulants (DOAC) are widely used alternatives to Vitamin-K antagonists (VKA). Women of reproductive age may become pregnant during anticoagulation and, while VKA carry an embryotoxic potential, the risk of DOAC embryopathy is unknown. As a result, some patients elect to terminate pregnancy for ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH16-04-0305
更新日期：2016-09-27 00:00:00
abstract：:We have studied 155 subjects, 48 normals, 36 diabetics without complications, 44 with complications and 27 patients with macroangiopathy. beta-Thromboglobulin (beta-TG) and platelet factor 4 (PF4) are elevated in the patients groups. There is no correlation between the plasma levels of beta-TG and the stages of either...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1984-12-29 00:00:00
abstract：:The endocannabinoid anandamide (AEA) has many neurovascular activities. However, it is not yet clear how AEA can be metabolized at the neurovascular interface, and how it can move through the vascular and the cerebral compartments. The results reported in this article show that isolated bovine brain microvessels, an e...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2006-01-01 00:00:00
abstract：:CD36, also known as GPIIIb or GPIV, is a main protein of the platelet membrane. Accumulating evidence implicates CD36 in the pathogenesis of atherosclerosis. CD36 binds to cholesteryl-hemisuccinate coupled to agarose. This binding may be of physiologic relevance since CD36 has a long extracellular hydrophobic region a...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1998-05-01 00:00:00
abstract：:The incidence of newly diagnosed malignancy is increased in patients with unexplained venous thromboembolism during the first year after a thromboembolic event in comparison to controls (odds ratio, 3.9-36). Extensive screening with computed tomography, endoscopy and tumor markers can identify most of these undetected...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：1997-07-01 00:00:00
abstract：:Thrombocytopenia occurred in 11.6% of 43 patients prospectively randomized to receive either bovine lung or porcine mucosal heparin. Four patients received bovine heparin and developed mild to moderate thrombocytopenia after eight to twelve days which resolved when therapy was stopped in one to six days. One patient r...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章,随机对照试验
doi：
更新日期：1980-02-29 00:00:00
abstract：:Bleeding time and platelet function tests were performed on 31 patients with progressive chronic renal failure (CRF) due to non-immunological (urological) causes, and compared with 22 healthy controls. Patients were classified as mild (plasma creatinine less than 300 mumol/l), moderate (300-600 mumol/l) or severe rena...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1988-08-30 00:00:00
abstract：:A technique has been developed to monitor the development of thrombin in freshly collected whole blood in the absence of anticoagulants. It is based on the centrifugal separation of the cellular components from subsamples of blood drawn from non-anticoagulated clotting whole blood which are diluted in buffer containin...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1994-07-01 00:00:00
abstract：:The endothelial protein C receptor (EPCR) is expressed by trophoblast cells. Mid-gestation pregnancy loss is described in animals with a haemochorial placenta lacking EPCR. The A6936G allele of the EPCR gene (PROCR) may be associated with lower EPCR densities on trophoblasts, but data are lacking for its effect on the...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH-09-04-0224
更新日期：2009-10-01 00:00:00
abstract：:alpha-Granule protein storage is important for producing platelets with normal haemostatic function. The low to undetectable levels of several megakaryocyte-synthesized alpha-granule proteins in normal plasma suggest megakaryocytes are important to sequester these proteins in vivo. alpha-Granule protein storage in vit...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH03-02-0125
更新日期：2003-11-01 00:00:00
abstract：:Three single nucleotide polymorphisms (-603A/G, -1322C/T, -1812C/T) and one deletion/insertion polymorphism (-1208D/I) are present in the tissue factor (TF) gene promoter sequence. These polymorphisms are in complete linkage disequilibrium, determining two haplotypes with almost equal frequency. The -603A/-1208D/-1322...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH03-09-0566
更新日期：2004-02-01 00:00:00
abstract：:Fibrin polymerisation is mediated by interactions between knobs 'A' and 'B' exposed by thrombin cleavage, and holes 'a' and 'b'. We demonstrated markedly delayed thrombin-catalysed fibrin polymerisation, through B:b interactions alone, of recombinant γD364H -fibrinogen with impaired hole 'a'. To determine whether reco...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH12-09-0684
更新日期：2013-02-01 00:00:00
abstract：:Atherosclerosis is at least in part an inflammatory disease. CD14 is an endotoxin receptor that after binding of lipopolysaccharides evokes endothelial activation and secretion of several cytokines. A polymorphism of CD14 has been associated with myocardial infarction. We evaluated the role of the -159 T/C polymorphis...
journal_title：Thrombosis and haemostasis
pub_type： 临床试验,杂志文章,多中心研究,随机对照试验
doi：10.1160/TH03-11-0668
更新日期：2004-05-01 00:00:00
abstract：:Megakaryocyte (MK)-derived miRNAs have been detected in platelets. Here, we analysed the expression of platelet and circulating miR-223, miR-26b, miR-126 and miR-140 that might be altered with their target mRNAs in type 2 diabetes mellitus (DM2). MiRNAs were isolated from leukocyte-depleted platelets and plasma sample...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH16-04-0322
更新日期：2017-02-28 00:00:00
abstract：:Syndecan-4 (ryudocan) is a cell-surface heparan sulfate proteoglycan, which plays an important role in a variety of biological functions including regulation of blood coagulation. cell adhesion, and cell growth. In this study, we measured plasma levels of syndecan-4 in patients with acute myocardial infarction using a...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2001-05-01 00:00:00
abstract：:The thrombin time of normal citrated plasma is progressively prolonged on incubation in open glass tubes at 37 degrees C. The phenomenon is dependent on the temperature and duration of incubation, on the pH, and on the concentration of calcium ions present. Platelet-rich citrated plasma fails to exhibit augmented anti...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1977-02-28 00:00:00
abstract：:In hereditary angioedema (HAE), normal C1-inhibitor (C1-INH) is low and the contact system activated. Recently, the findings of a tissue factor mutant selectively deficient in promoting the conversion of FVII to FVIIa, but with retained cofactor for FVIIa, made it possible to examine reliably the pre-existing content ...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1995-10-01 00:00:00
abstract：:Mice with a total deficiency in blood coagulation Factor X (FX) were generated by targeted replacement of an 18-kb fragment of the FX gene, comprising all exons encoding the mature FX protein, with a neo(r) cassette. The genotype distribution among the offspring from heterozygous breeding pairs suggested that FX defic...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2000-02-01 00:00:00
abstract：:Vitamin K-dependent proteins are currently purified from pooled human plasma or produced in mammalian cell culture systems by recombinant DNA technology. Transgenic animals may provide an additional expression system for the production of these therapeutic proteins. Mice have been used to test hybrid genes which direc...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章,评审
doi：
更新日期：1997-07-01 00:00:00
abstract：:Washed human platelets have been labeled with either 32Pi or glycerol-1-14C and the distribution of the label in the phospholipids determined. 32Pi was introduced primarily into polyphosphoinositides, i.e. di- and triphosphoinositide, whereas the label from glycerol which indicates de novo synthesis of lipid molecules...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1976-04-30 00:00:00
abstract：:We have investigated the effect of a new Leu196Pro mutation, identified in the MIDAS-like domain of the beta3 integrin subunit in a patient with type II Glanzmann thrombasthenia, on beta3 integrin receptor function. Expression of the mutant beta3Pro196 subunit in CHO cells, either associated with recombinant human alp...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：2001-12-01 00:00:00
abstract：:The synthetic peptide SFLLRNPNDKYEPF, identical in sequence to the new amino-terminus of the thrombin receptor generated following cleavage of thrombin, acts a thrombin receptor agonist/activating peptide (TRAP). In this study, Northern blot analysis showed that cultured human vascular smooth muscle cells (HVSMC) expr...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1995-11-01 00:00:00
abstract：:D-dimer, a marker of fibrin turnover, exhibits many interesting properties as a biological marker of thrombosis. Some of the properties of D-dimer might also be used to provide additional information about patients with heart failure. In this study, we evaluate the prognostic information acquired from D-dimer concerni...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：10.1160/TH04-05-0278
更新日期：2004-12-01 00:00:00
abstract：: ...
journal_title：Thrombosis and haemostasis
pub_type： 社论
doi：10.1055/s-0038-1676073
更新日期：2018-12-01 00:00:00
abstract：:Causative mutations in the factor VIII gene of seven unrelated patients with severe haemophilia A were identified using the mutation screening procedure conformation sensitive gel electrophoresis (1) and characterised by direct sequencing. Female family members of all patients had requested either carrier status deter...
journal_title：Thrombosis and haemostasis
pub_type： 杂志文章
doi：
更新日期：1998-04-01 00:00:00
