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  2. Jul 29, 2022 · Also, observations of patients with von Willebrand disease (VWD) could potentially be useful. Despite the heterogeneity of malignant diseases, can we get any information about whether VWD patients are at lower/higher risk of developing cancer? Alternatively, is cancer progression different in patients with VWD?

    • 10.1111/jth.15810
    • 2022/10
    • What Every Physician Needs to Know
    • Are You Sure Your Patient Has VWD? What Should You Expect to find?
    • Beware of Other Conditions That Can Mimic VWD
    • More Definitive Therapies?
    • What Other Therapies Are Helpful For Reducing Complications?
    • What Should You Tell The Patient and The Family About Prognosis?
    • What If Scenarios
    • Pathophysiology
    • What’s The Evidence?

    Von Willebrand factor (VWF) is a huge multimeric protein comprised of identical 280,000 Dalton monomeric subunits. VWF is critical in the initiation of hemostasis. VWF multimers are produced and secreted by endothelial cells (and platelets) and, because they bind to platelet glycoprotein (GP) I receptors, induce platelet adherence to long VWF multi...

    VWD is usually an inherited bleeding disorder. Only rarely is it acquired. A family history of bleeding, especially affecting both males and females, suggests VWD. It is a mild bleeding disorder in many patients. Diagnostic evaluation is often initiated to determine the cause of easy bruising, excessive bleeding with dental extractions or surgery, ...

    Hemophilia A and either quantitative or qualitative platelet disorders can resemble VWD clinically. Hemophilia A is an X-linked deficiency of FVIII that primarily affects males. VWD is an autosomal disorder (discussed further in “Pathophysiology”). Platelet-type (or “pseudo”-) VWD is a gain-of-function defect in platelet GP Ib with similar clinical...

    Humate-P is a concentrate that contains all VWF multimers and FVIII, and is given either by rapid IV infusion or (sometimes) continuous infusion. The product is labeled for both RCoF and FVIII dosing. (Other VWF-containing products are described and compared by Favoloro 2016 in the references.) For a bleeding episode, Humate-P is usually administer...

    The anti-fibrinolytics, aminocaproic acid (Amicar; 50 mg/kg [up to a maximum of 5 grams] given 4 times daily) or tranexamic acid (25 mg/kg given 3 times daily) are especially useful in the management of oral/dental bleeding. Estrogen or oral contraceptive pills can sometimes be effective in the management of bleeding (including menorrhagia) in wome...

    The prognosis for most patients with VWD is favorable, provided their bleeding is treated and prevented appropriately. Many individuals with VWD are followed routinely at a hemophilia treatment center. Individuals with VWD should be discouraged from participating in high-impact sports/activities (football, boxing, rugby, wrestling, rodeo-type event...

    Most cases of VWD are inherited. Rarely, however, VWD is acquired in association with: (1) multiple myeloma, lymphoproliferative or myeloproliferative disorders (acquired VWD caused by anti-VWF autoantibodies to, or cell absorption of, large VWF multimers); (2) hypothyroidism (caused by decreased VWF synthesis); or (3) serious cardiac conditions as...

    The gene encoding the VWF monomer is on chromosome #12. Expression of the mRNA for the VWF monomer is translated into a 280,00 Dalton protein that contains a series of different domains. These include contiguous non-identical regions (A domains) that: (a) bind to platelet GP Ib (the A1 domain); (b) contain the peptide cleavage site for ADAMTS-13, t...

    James, AH, Manco-Johnson, MJ, Yawn, BP, Dietrich, JE, Nichols, WL. “Von Willebrand disease: key points from the 2008 National Heart, Lung, and Blood Institute guidelines”. Obstet Gynecol. vol. 114. 2009. pp. 674-8. (Summary of management in Ob-Gyn journal.) Ruggeri, ZM, Ware, J. “von Willebrand factor”. FASEB J. vol. 7. 1993. pp. 308-16. (Classic p...

  3. In fact, VTE has been reported in up to 20% of cancer patients making it one of the leading causes of death for cancer patients. 9 Moreover, growing evidence suggests that this crosstalk between coagulation pathways and cancer is of direct clinical relevance, contributing not only to cancer associated thrombosis (CAT) but to cancer progression ...

    • Sean Patmore, Sukhraj Pal Singh Dhami, Jamie M. O’Sullivan
    • 2020
  4. Apr 29, 2021 · In conclusion, yes, there are some efficient treatments for VWD, but, no, this does not mean that we should stop trying to improve these treatments with the goals of reaching superior efficacy, improving quality of life of the patients, and finally, curing the disease.

    • Cécile V Denis, Sophie Susen, Peter J Lenting
    • 2021
  5. What is von Willebrand disease? Discover the symptoms and stages, as well as the treatments and research being pioneered at the OSUCCC – James.

  6. May 29, 2023 · There’s no cure for von Willebrand disease, but it can be treated and/or managed. A key to managing this disorder is to reduce the risk of bleeding before it starts. This means avoiding...

  7. Aug 28, 2023 · Acquired von Willebrand disease prevalence is unknown but may represent 1% to 5% of all von Willebrand disease. Its prevalence is higher in certain groups. For example, it has been reported in up to 20% of malignancies and up to 100% of certain high flow states such as extracorporeal membrane oxygenation (ECMO) and metallic cardiac valves.

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