How I treat patients with von Willebrand disease

Mannucci, Pier Mannuccio

doi:10.1182/blood.v97.7.1915

Cited by 146 publications

(131 citation statements)

References 42 publications

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“…In Type 2N, relatively high levels of FVIII are observed following DDAVP, but released FVIII circulates for a shorter time period in patient plasma because the stabilizing effect of VWF is impaired. Finally, patients with Type 3 VWD are usually unresponsive to DDAVP [11].…”

Section: Desmopressin In the Treatment Of Inherited Bleeding Disordersmentioning

confidence: 99%

The use of desmopressin as a hemostatic agent: A concise review

Franchini

2007

American J Hematol

126

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Desmopressin, a synthetic derivative of the antidiuretic hormone vasopressin, is the treatment of choice for most patients with von Willebrand disease and mild hemophilia A. Moreover, the compound has been shown to be useful in a variety of inherited and acquired hemorrhagic conditions, including some congenital platelet function defects, chronic liver disease, uremia, and hemostatic defects induced by the therapeutic use of antithrombotic drugs such as aspirin and ticlopidine. Finally, desmopressin has been used as a blood saving agent in patients undergoing operations characterized by large blood loss and transfusion requirements, but studies suggest that this is not as effective as other methods. This review briefly summarizes the current clinical indications on the use of desmopressin as a hemostatic agent. Am. J. Hematol., 2007. © 2007 Wiley‐Liss, Inc.

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Section: Desmopressin In the Treatment Of Inherited Bleeding Disordersmentioning

confidence: 99%

The use of desmopressin as a hemostatic agent: A concise review

Franchini

2007

American J Hematol

126

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“…DDAVP, if required during pregnancy, is generally thought to be safe for mother and foetus [68,69]. At the time of childbirth, DDAVP must be used with caution, if at all.…”

Section: Management Of Pregnancy and Childbirthmentioning

confidence: 99%

Women and bleeding disorders

James

2010

Haemophilia

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Summary. While women are rarely affected by haemophilia, they are equally as likely as men to have other bleeding disorders. Menorrhagia, or heavy menstrual bleeding, is the most common symptom that they experience. Not only is menorrhagia more prevalent among women with bleeding disorders, but bleeding disorders are more prevalent among women with menorrhagia. Although menorrhagia is the most common reproductive tract manifestation of a bleeding disorder, it is not the only manifestation. Women with bleeding disorders appear to be at an increased risk of developing haemorrhagic ovarian cysts and possibly endometriosis. Women suspected of having a bleeding disorder or being a carrier of haemophilia should be offered diagnostic testing before getting pregnant to allow for appropriate preconception counselling and pregnancy management. During pregnancy, women with bleeding disorders may be at an increased risk of bleeding complications. At the time of childbirth, women with bleeding disorders appear to be more likely to experience postpartum haemorrhage, particularly delayed or secondary postpartum haemorrhage. As women with bleeding disorders grow older, they may be more likely to manifest gynaecological conditions which present with bleeding. Women with bleeding disorders are more likely to undergo a hysterectomy and are more likely to have the operation at a younger age. While women with bleeding disorders are at risk for the same obstetrical and gynaecological problems that affect all women, women with bleeding disorders are disproportionately affected by conditions that manifest with bleeding. Optimal management involves the combined expertise of haemostasis experts and obstetrician-gynaecologists.

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“…Nontransfusional therapies such as antifibrinolytics (ε-aminocaproic acid or tranexamic acid), 1,25,56,66 -68,73,78 -86 desmopressin, 21,22,25,35,36,68,77,80,[87][88][89][90][91] and vitamin K 92-94 may be used for the treatment or prevention of bleeding in patients with bleeding disorders (Table 6) and occasionally as sole hemostatic agents for the treatment of mild mucosal bleeding or for hemostatic coverage during minor (eg, dental) procedures, thus sparing the patient exposure to transfusional therapies. Nontransfusional therapies can also be used as an adjunct to transfusional therapies in cases of more severe bleeding or for hemostatic coverage during major surgery.…”

Section: Nontransfusional Hemostatic Therapiesmentioning

confidence: 99%

Identification and Basic Management of Bleeding Disorders in Adults

Kruse‐Jarres

Singleton

Leissinger

2014

The Journal of the American Board of Family Medicine

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Adults with bleeding disorders may present to their family physician with minor bleeding symptoms or hematologic laboratory abnormalities discovered during evaluation for surgery or another purpose. Identifying the small proportion of adults who have an underlying bleeding disorder as the cause for such signs or symptoms may be challenging. In cases of asymptomatic hematologic laboratory abnormalities, the particular abnormality should narrow down the potentially affected hemostatic component(s), ideally streamlining subsequent investigation. In patients presenting with bleeding symptoms, a thorough history and physical examination are critical for first identifying bleeding as pathologic, then performing the appropriate diagnostic evaluation after excluding identifiable causes. Knowledge of the pathophysiologic processes contributing to impaired hemostasis in any given bleeding disorder ensures proper treatment and avoids therapies that are unnecessary or even contraindicated. Management is further determined by bleeding phenotype and, for invasive procedures, the anticipated risk for bleeding. Consultation with a hematologist may facilitate proper evaluation and treatment, particularly in adults with rare bleeding disorders or no identifiable cause for bleeding. This article reviews the diagnostic approach to hematologic laboratory abnormalities and abnormal bleeding in adults, as well as basic preventive care and hemostatic management of adults with bleeding disorders. (J Am Board Fam Med 2014;27:549 -564.)

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How I treat patients with von Willebrand disease

Cited by 146 publications

References 42 publications

The use of desmopressin as a hemostatic agent: A concise review

The use of desmopressin as a hemostatic agent: A concise review

Women and bleeding disorders

Identification and Basic Management of Bleeding Disorders in Adults

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