Aspects of the laboratory identification of von Willebrand disease in women.


:The increased prevalence of the laboratory diagnosis of von Willebrand disease (vWD) in women presenting with menorrhagia has raised concerns regarding certain specifics in vWD testing in women, including when vWD testing should be done in relation to menses and whether testing should be done while the patient is not taking an oral contraceptive (OC). These concerns have been based on historical reports that vWF and factor (F) VIII:coagulant activity levels can decrease during menses and conceivably increase the probability of diagnosing vWD when the patient is menstruating, whereas hormone therapy can increase the vWF levels and conceivably mask the diagnosis of vWD. Historically, the reports of a decrease in vWF levels during menstruation have been in a relatively small total number of patients; this has not been confirmed in two recent studies. In one study of 95 normal menstruating females sampled serially at days 4 to 7, 11 to 15, and 21 to 28, there was no variation. In another study of 40 volunteers, by cross-sectional analysis there was no difference. However, interestingly in that study, longitudinal analysis of samples showed a decrease in vWF antigen during menstruation. In another recently published study, using cross-sectional analysis, the lowest levels of vWF were found on days 1 through 4, whereas the highest were identified on days 9 through 10. Conceivably, these groups were more finely divided than were those in the other studies. In summary, in light of these conflicting results, recommendations for testing exclusively during menses cannot be made. At this point, it is not unreasonable to suggest that hematologists and gynecologists sample at least once at the time of menstrual bleeding when the diagnosis of vWD in a menstruating female is suspected. Consequently, sampling during the menstrual cycle may likely capture the lowest levels of FVIIIC and vWF antigen. Regarding testing while the patient is taking an OC, present preparations do not contain supraphysiological doses of estrogen and are unlikely to affect the laboratory diagnosis of vWD. These studies primarily have been in controls. Therefore, prospective studies in vWD women of menstrual variation of the vWF levels and the impact of OCs on vWF levels are in order before specific recommendations can be made regarding the timing of testing and whether patients should be tested while the patient is not taking an OC. Other aspects regarding race, age, preanalytical variables, and pregnancy potentially affecting the laboratory diagnosis of vWD are also discussed in this review.


Semin Thromb Hemost


Kouides PA




Has Abstract


2006-07-01 00:00:00












  • Thromboembolic disease in surgery for malignancy-rationale for prolonged thromboprophylaxis.

    abstract::Patients undergoing surgery for malignancy are at increased risk of initial and recurrent venous thromboembolism (VTE). Several factors have been found to increase the risk of deep vein thrombosis (DVT) in cancer patients both during the first days after the operation and after discharge from hospital. Although, in ge...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Khushal A,Quinlan D,Alikhan R,Gardner J,Bailey C,Cohen A

    更新日期:2002-12-01 00:00:00

  • Management of bleeding complications of hematologic malignancies.

    abstract::Persons with hematologic malignancies bleed for a variety of reasons, including alterations in platelet function and numbers, clotting factor deficiencies, circulating anticoagulants, and defects in vascular integrity. The management of bleeding begins with a full characterization of the hemostatic defect. Vitamin K d...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Green D

    更新日期:2007-06-01 00:00:00

  • Case studies of HIT in cardiovascular medicine.

    abstract::We present three clinical cases that illustrate some of the key features of the diagnosis and management of immune-mediated heparin-induced thrombocytopenia (HIT). The importance of having a high clinical suspicion for HIT in the appropriate clinical setting is emphasized. Early therapeutic decisions should be based o...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Smith KJ,Call JT,Sane DC

    更新日期:2004-06-01 00:00:00

  • Women with thrombophilia: assessing the risks for thrombosis with oral contraceptives or hormone replacement therapy.

    abstract::Women with inherited or acquired thrombophilia are at increased risk for venous thromboembolism (VTE) when they use oral contraceptives (OCs) of either the second or third generation. For women who are heterozygous for Factor V Leiden, the risk is probably 28 to 50 of 10,000 women-years compared to 2 to 5 of 10,000 ye...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Waselenko JK,Nace MC,Alving B

    更新日期:1998-01-01 00:00:00

  • Coronavirus (COVID-19), Coagulation, and Exercise: Interactions That May Influence Health Outcomes.

    abstract::The proinflammatory cytokine storm associated with coronavirus disease 2019 (COVID-19) negatively affects the hematological system, leading to coagulation activation and endothelial dysfunction and thereby increasing the risk of venous and arterial thrombosis. Coagulopathy has been reported as associated with mortalit...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Zadow EK,Wundersitz DWT,Hughes DL,Adams MJ,Kingsley MIC,Blacklock HA,Wu SSX,Benson AC,Dutheil F,Gordon BA

    更新日期:2020-10-01 00:00:00

  • Classic and Global Hemostasis Testing in Pregnancy and during Pregnancy Complications.

    abstract::Pregnancy is associated with a significant procoagulant shift in the hemostatic system balance as well as other metabolic changes. Pregnancy can thereby provoke manifestation of otherwise dormant disorders of hemostasis (e.g., thrombophilia), or even cause new, pregnancy-specific disorders (e.g., HELLP syndrome). Appl...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Ataullakhanov FI,Koltsova EM,Balandina AN,Serebriyskiy II,Vuimo TA,Panteleev MA

    更新日期:2016-10-01 00:00:00

  • Massive posttraumatic bleeding: epidemiology, causes, clinical features, and therapeutic management.

    abstract::Bleeding typically results as a consequence of derangements of primary hemostasis, secondary hemostasis, or both, and can be dramatically amplified by the presence of other predisposing conditions, especially inherited bleeding disorders. Life-threatening hemorrhages are, however, almost exclusively caused by penetrat...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Lippi G,Favaloro EJ,Cervellin G

    更新日期:2013-02-01 00:00:00

  • Comparison of recombinant human thrombin and plasma-derived human alpha-thrombin.

    abstract::Bleeding can be a serious complication of surgery, and topical thrombin is widely used as an adjunct to hemostasis in diverse surgical settings. The potent hemostatic properties of thrombin derive from its ability to activate platelets directly to aggregate and adhere to damaged vessels and to catalyze the formation s...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Bishop PD,Lewis KB,Schultz J,Walker KM

    更新日期:2006-04-01 00:00:00

  • Protein C and the endothelium.

    abstract::With the continued accumulation of clinical and animal studies, it is becoming abundantly clear that the protein C anticoagulant pathway plays a critical role in the regulation of coagulation. Investigations also indicate that this pathway is intimately involved in the interaction of the coagulation and inflammatory s...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Esmon NL,Esmon CT

    更新日期:1988-04-01 00:00:00

  • Thrombolytic activity of defibrotide against old venous thrombi.

    abstract::The age of the thrombus is probably a very important determinant of the outcome of thrombolysis. The clinical potential for rapidly dissolving thrombi by thrombolytic therapy is considerable because restoration of the blood flow can rescue the jeopardized district served by the occluded vessel such as for myocardial i...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Niada R,Porta R,Pescador R,Mantovani M,Prino G

    更新日期:1989-10-01 00:00:00

  • Management strategies for optimal control of anticoagulation in patients with atrial fibrillation.

    abstract::Most patients with atrial fibrillation need anticoagulant treatment with vitamin K antagonists for prevention of thromboembolism, in particular ischemic stroke. Many studies show the efficacy of this treatment but also that it is difficult to keep patients who use vitamin K antagonists in the proper treatment range. B...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Levi M,de Peuter OR,Kamphuisen PW

    更新日期:2009-09-01 00:00:00

  • Latent heparanase facilitates VLA-4-mediated melanoma cell binding and emerges as a relevant target of heparin in the interference with metastatic progression.

    abstract::Heparanase is an endo-β-glucuronidase that enzymatically cleaves heparan sulfates (HS) and heparan sulfate proteoglycan (HSPG) structures. Heparanase expression levels by tumors were correlated with cell invasion, angiogenic activity, and poor prognosis. Heparanase can also possess pro-tumorigenic effects independent ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Gerber U,Hoß SG,Shteingauz A,Jüngel E,Jakubzig B,Ilan N,Blaheta R,Schlesinger M,Vlodavsky I,Bendas G

    更新日期:2015-03-01 00:00:00

  • Inhibitor development in previously untreated patients with hemophilia A: a prospective long-term follow-up comparing plasma-derived and recombinant products.

    abstract::In order to assess inhibitor development in previously untreated patients (PUPs) with severe (factor VIII [FVIII]<1%) and moderate (FVIII 1 to 5%) hemophilia A, a prospective study was initiated in 1976. During the 23-year study period, 72 hemophiliacs were frequently exposed prophylactically or on demand to plasma-de...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Kreuz W,Ettingshausen CE,Zyschka A,Oldenburg J,Saguer IM,Ehrenforth S,Klingebiel T

    更新日期:2002-06-01 00:00:00

  • Anticoagulants and extracorporeal circuits.

    abstract::Anticoagulants are pivotal to achieve circulation in extracorporeal circuits. In this review we discuss several anticoagulants in clinical use or in the preclinical phase. In hemodialysis the low-molecular-weight heparins (LMWHs) appear to be as effective and safe as standard heparin (SH). The main advantages of LMWHs...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Beijering RJ,ten Cate H,Nurmohamed MT,ten Cate JW

    更新日期:1997-01-01 00:00:00

  • Selectins in the HIT syndrome: pathophysiologic role and therapeutic modulation.

    abstract::The pathophysiology of heparin-induced thrombocytopenia (HIT) is now known to be a complex process which involves platelets, vascular endothelium, and leukocytes. The activation products from these sites also contribute to the activation of coagulation and to the fibrinolytic deficit. While many of the markers of hemo...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Fareed J,Walenga JM,Hoppensteadt DA,Jeske WP,Lietz H,Ahmad S,Callas D,Messmore HL,Haas S

    更新日期:1999-01-01 00:00:00

  • Identification of p.W246L as a novel mutation in the GP1BA gene responsible for platelet-type von Willebrand disease.

    abstract::Platelet-type von Willebrand disease (PT-VWD) and type 2B von Willebrand disease (2B-VWD) are rare bleeding disorders characterized by increased ristocetin-induced platelet aggregation (RIPA) at low concentrations of ristocetin. Diagnosis of either condition is not easy and the differential diagnosis between the two e...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Woods AI,Sanchez-Luceros A,Bermejo E,Paiva J,Alberto MF,Grosso SH,Kempfer AC,Lazzari MA

    更新日期:2014-03-01 00:00:00

  • Use of D-dimer assays in the diagnosis of venous thrombosis.

    abstract::In the course of fibrin formation, the D-domains of adjacent fibrin molecules within the fibrin polymer are covalently linked by factor XIIIa, leading to the formation of a D-domain dimer. Proteolysis of this cross-linked fibrin generates fibrin fragments D-dimer and E as terminal products. Fragment D-dimer therefore ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Dempfle CE

    更新日期:2000-01-01 00:00:00

  • The Complications of Vascular Access in Hemodialysis.

    abstract::Complications related to hemodialysis vascular access continue to have a major impact on morbidity and mortality. Vascular access dysfunction is the single most important factor that determines the quality of dialysis treatment. Vascular access stenosis is a common complication that develops in a great majority of pat...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Masud A,Costanzo EJ,Zuckerman R,Asif A

    更新日期:2018-02-01 00:00:00

  • Management of Patients Taking Oral Anticoagulants Who Need Urgent Surgery for Hip Fracture.

    abstract::The number of hip fractures in anticoagulated patients is predicted to increase, due to people living longer. However, evidence regarding urgent perioperative management of elderly patients with hip fracture who take oral anticoagulants (vitamin K antagonists or direct oral anticoagulants) is scarce. In this article, ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Grandone E,Ostuni A,Tiscia GL,Marongiu F,Barcellona D

    更新日期:2019-03-01 00:00:00

  • Protective Effects of Statins Administration in European and North American Patients Infected with COVID-19: A Meta-analysis.

    abstract::Severe acute respiratory syndrome coronavirus 2 has spread rapidly throughout the world, becoming an overwhelming global health emergency. The array of injuries caused by this virus is broad and not limited to the respiratory system, but encompassing also extensive endothelial and systemic tissue damage. Since statins...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Onorato D,Pucci M,Carpene G,Henry BM,Sanchis-Gomar F,Lippi G

    更新日期:2021-01-22 00:00:00

  • Role of Tissue Factor in the Coagulation Network.

    abstract::It is generally accepted that the initial event in coagulation and intravascular thrombus formation is the exposure of the flowing blood to cell-surface protein, such as tissue factor (TF). Vascular injury and/or atherosclerotic plaque complication is responsible for this exposure, leading to clinical manifestations s...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Cimmino G,Ciccarelli G,Golino P

    更新日期:2015-10-01 00:00:00

  • Mechanisms linking tumor cell-associated procoagulant function to tumor dissemination.

    abstract::There is a persuasive body of evidence suggesting that tissue factor (TF) is a major determinant of tumor progression. In addition to its "traditional" function as the initiator of hemostasis, TF may support tumor progression through signaling mechanisms involving either direct signal transduction through the TF cytop...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Palumbo JS

    更新日期:2008-03-01 00:00:00

  • Factor XIII in chronic inflammatory bowel diseases.

    abstract::Severe acute inflammation in chronic inflammatory bowel disease is associated with large wound areas and ulcerations that show spontaneous hemorrhage or marked friability. Therefore, an enormous potential of hemostasis and wound healing is required. Coagulation studies demonstrate a deficiency of factor XIII that is i...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Lorenz R,Olbert P,Born P

    更新日期:1996-01-01 00:00:00

  • Platelet dysfunction in renal failure.

    abstract::Patients with end-stage renal disease suffer from complex hemostatic disorders. Uremic patients show a bleeding diathesis that is mainly due to abnormalities of primary hemostasis; in particular, platelet dysfunction and impaired platelet-vessel wall interaction. However, despite decreased platelet function, these pat...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Boccardo P,Remuzzi G,Galbusera M

    更新日期:2004-10-01 00:00:00

  • New developments in lung endothelial heterogeneity: Von Willebrand factor, P-selectin, and the Weibel-Palade body.

    abstract::Quiescent pulmonary endothelium establishes an antithrombotic, anti-inflammatory surface that promotes blood flow. However, the endothelium rapidly responds to injury and inflammation by promoting thrombosis and enabling the directed transmigration of inflammatory cells, such as neutrophils, into the alveolar airspace...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Ochoa CD,Wu S,Stevens T

    更新日期:2010-04-01 00:00:00

  • Acute pulmonary embolism after an earthquake in Japan.

    abstract::There have been no reports on acute pulmonary embolism (APE) after earthquakes. Our aim was to clarify the actual the occurrence of APE following the 2004 Mid Niigata Prefecture earthquake in Japan, and to assess the risk factors for APE after the event. We sent questionnaires to 122 hospitals in the Niigata Prefectur...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,多中心研究


    authors: Sakuma M,Nakamura M,Hanzawa K,Kobayashi T,Kuroiwa M,Nakanishi N,Miyahara Y,Tanabe N,Yamada N,Kuriyama T,Kunieda T,Sugimoto T,Nakano T,Shirato K

    更新日期:2006-11-01 00:00:00

  • International survey on D-dimer test reporting: a call for standardization.

    abstract::D-dimer is the biochemical gold standard for diagnosing a variety of thrombotic disorders, but result reporting is heterogeneous in clinical laboratories. A specific five-item questionnaire was developed to gain a clear picture of the current standardization of D-dimer test results. The questionnaire was opened online...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Lippi G,Tripodi A,Simundic AM,Favaloro EJ

    更新日期:2015-04-01 00:00:00

  • Vitamin K-Dependent Protein S: Beyond the Protein C Pathway.

    abstract::Protein S is a vitamin K-dependent plasma glycoprotein circulating in plasma at a concentration of around 350 nM. Approximately 60% of protein S in human plasma is bound to the complement regulatory protein C4b-binding protein (C4BP) in a high-affinity, high-molecular-weight complex. Protein S in plasma has multiple a...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Dahlbäck B

    更新日期:2018-03-01 00:00:00

  • Shear-induced pathway of platelet function in cardiac surgery.

    abstract::The contribution of platelet dysfunction to the impaired hemostasis after cardiac surgery remains to be established, because there is no sensitive method to assess platelet function. Measurement of the shear-induced pathway of platelet function, an important mechanism in inducing hemostasis, became possible by a novel...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章


    authors: Tabuchi N,Tigchelaar I,van Oeveren W

    更新日期:1995-01-01 00:00:00

  • Type 2B von Willebrand Disease: A Matter of Plasma Plus Platelet Abnormality.

    abstract::Type 2B von Willebrand disease (VWD2B) is a rare, autosomal-dominant inherited bleeding disorder, characterized by an enhanced ristocetin-induced platelet aggregation in platelet-rich plasma and often with variable degree of thrombocytopenia and loss of high-molecular-weight multimers von Willebrand factor (VWF). All ...

    journal_title:Seminars in thrombosis and hemostasis

    pub_type: 杂志文章,评审


    authors: Castaman G,Federici AB

    更新日期:2016-07-01 00:00:00