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Psuedo von Willebrand disease in pregnancy can be confused with a number of other differential diagnoses, such as gestational thrombocutopenia, idiopathatic thrombocytopenia, thrombotic thrombocytopenic purpura and pre-eclampsia; all need consideration during investigations even in a case such as this where the diagnosis of platelet type von Willebrand disease was known before pregnancy.
- Neetu Grover, Vincent Boama, Munazzah Rifat Chou
May 01, 2017 · A 37-year-old woman 28 weeks into her first pregnancy is referred to hematology clinic given a history of von Willebrand disease (vWD) diagnosed during her teenage years, after presenting with excessive menstrual bleeding requiring oral iron supplementation.
- Caroline Berube, Brian Dietrich
Von Willebrand disease (VWD) occurs with equal frequency among men and women, affecting up to 1% of the general population.1,2However, women are more likely to experience symptoms of VWD because of...
In a CDC survey of women with VWD: [Read articleexternal icon] 1. There was an average of 16 years between the onset of their bleeding symptoms and diagnosis of a bleeding disorder. 2. Women reported an average of 6 bleeding symptoms before a diagnosis of VWD was made. Common symptoms included menorrhagia, bruising, nosebleeds, and bleeding from surgery, injury, and delivery. Menorrhagia was the most commonly reported symptom. 3. To obtain the diagnosis of VWD, tests were carried out on average twice (range 1–20 times). 4. 38% of women reported that they were first diagnosed by a Hemophilia Treatment Center (HTC) doctor. Among women diagnosed by a provider other than an HTC doctor, 42% were diagnosed by a hematologist, 28% by an internist or a family doctor and 19% by a gynecologist.
In a CDC study of 102 women with all types of VWD compared to 88 controls: [Read articleexternal icon] 1. The most commonly reported bleeding symptoms among women with VWD were: 1.1. Heavy menstrual bleeding (95%) 1.2. Bleeding after minor injuries (92%) 1.3. Excessive gum bleeding (76%) 2. 74% of women with VWD reported excessive bleeding from several sites (e.g. nose, gum and uterus) or following several procedures or injuries (e.g. dental, surgical, childbirth and minor injury) compared to 6% of controls. 3. 41% of women with VWD reported a diagnosis of migraine headaches compared with 13% of controls. 4. 37% of women with VWD reported a diagnosis of arthritis compared with 15% of controls. 5. 37% of women with VWD compared with 10% of controls indicated that their menstrual period limited routine work, social activities and had a negative effect on life. 6. More women with VWD than controls in this study had undergone hysterectomy (25% vs. 9%). In studies of 42 men and 38 women...Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand’s disease. Blood. 1987 Feb;69(2):454-9.Werner EJ, Broxson EH, Tucker EL, et al. Prevalence of von Willebrand disease in children: a multiethnic study. J Pediatr 1993 Dec; 123(6): 893-8.Ragni MV, FA Bontempo, C Hassett. Von Willebrand disease and bleeding in women. Haemophilia 1999; 5:313-317.Ziv O, Ragni MV. Bleeding manifestations in males with von Willebrand disease. Haemophilia 2004; 10:162-8.
- Side effects
Von Willebrand disease is a bleeding disorder. Its caused by a deficiency of von Willebrand factor (VWF). This is a type of protein that helps your blood to clot. Von Willebrand is different from hemophilia, another type of bleeding disorder. Bleeding happens when one of your blood vessels breaks. Platelets are a type of cell that circulates in your blood and clumps together to plug broken blood vessels and stop bleeding. VWF is a protein that helps platelets clump together, or clot. If your levels of functional VWF are low, your platelets wont be able to clot properly. This leads to prolonged bleeding. A genetic mutation causes von Willebrand disease. The type of von Willebrand disease that you have depends on whether one or both of your parents have passed a mutated gene on to you. For example, you can only develop type 3 Von Willebrand if youve inherited a mutated gene from both of your parents. If youve only inherited one copy of the mutated gene, youll develop type 1 or 2 von Willebrand disease.
According to the Centers for Disease Control and Prevention, von Willebrand disease affects up to 1 percent of the general population in the United States. Men and women develop von Willebrand disease at the same rate. But women are more likely to experience symptoms and complications due to the increased risk of bleeding during menstruation, pregnancy, and childbirth.
If you have type 2 von Willebrand disease, you have normal levels of VWF but it wont work properly due to structural and functional defects. Type 2 is divided into subtypes, including types: Your doctor will ask you questions about your personal and family history of abnormal bruising and bleeding. Type 3 tends to be the easiest to diagnose. If you have it, youll likely have a history of severe bleeding starting early in life. Along with taking a detailed medical history, your doctor may also use laboratory tests to check for abnormalities in your VWF levels and function. They may also check for abnormalities in clotting factor VIII, which can cause hemophilia. Your doctor can also use blood tests to learn how well your platelets function. Your doctor will need to collect a sample of your blood to conduct these tests. Then, theyll send it to a laboratory for testing. Because of the specialized nature of these tests, it may take up to two or three weeks to receive your results.
Type 3 is the most dangerous type of von Willebrand disease. If you have this type, your body wont produce any VWF. As a result, your platelets wont be able to clot. This will put you at risk of severe bleeding thats difficult to stop.
If you have von Willebrand disease, your symptoms will vary depending on which type of the disease you have. The most common symptoms that occur in all three types include: Type 3 is the most severe form of the condition. If you have this type, you have no VWF in your body. This makes episodes of bleeding difficult to control. It also raises your risk of internal bleeding, including bleeding in your joints and gastrointestinal system.
If you have von Willebrand disease, your treatment plan will vary, depending on the type of condition you have. Your doctor may recommend several different treatments. Your doctor may prescribe desmopressin (DDAVP), which is a drug recommended for types 1 and 2A. DDAVP stimulates the release of VWF from your bodys cells. Common side effects include a headache, low blood pressure, and fast heart rate. Your doctor may also recommend replacement therapy, using Humate-P or Alphanate Solvent Detergent/Heat Treated (SD/HT). These are two types of biologics, or genetically engineered proteins. Theyre developed from human plasma. They can help replace the VWF thats absent or working improperly in your body. To treat minor bleeding from small capillaries or veins, your doctor may recommend applying Thrombin-JMI topically. They may also apply Tisseel VH topically after you undergo surgery, but it wont stop heavy bleeding.
These replacement therapies arent identical and you shouldnt use them interchangeably. Your doctor may prescribe Humate-P if you have type 2 von Willebrand disease and are unable to tolerate DDAVP. They may also prescribe it if you have a severe case of type 3 von Willebrand disease.
Common side effects of replacement therapy with Humate-P and Alphanate SD/HT include chest tightness, rash, and swelling.
Aminocaproic acid and tranexamic acid are drugs that help steady clots formed by platelets. Doctors often prescribe them to people who are undergoing invasive surgery. Your doctor may also prescribe them if you have type 1 von Willebrand disease. Common side effects include nausea, vomiting, and clot complications.
If you have any form of Von Willebrand disease, its important to avoid drugs that can increase your risk of bleeding and complications. For example, avoid aspirin and nonsteroidal anti-inflammatory drugs, such as ibuprofen and naproxen. No matter what type of von Willebrand disease you have, you should let your healthcare providers know about it, including your dentist. They may need to adjust their procedures to lower your risk of bleeding. You should also let trusted family members and friends know about your condition in case you have an unexpected accident or need life-saving surgery. They can share important information about your condition with your healthcare providers.
Most people who have type 1 von Willebrand disease are able to live normal lives with only mild bleeding issues. If you have type 2, youre at an increased risk of experiencing mild to moderate bleeding and complications. You may experience worse bleeding in the case of infection, surgery, or pregnancy. If you have type 3, youre at risk of severe bleeding, as well as internal bleeding.
- Lydia Krause
Von Willebrand disease (VWD) is a bleeding disorder in which your blood has trouble clotting. It is inherited, meaning it is passed down from parents to their children. People with von Willebrand disease sometimes experience heavier-than-normal bleeding from injury, surgery, and, in women, menstrual flow and childbirth.
Von Willebrand disease (VWD) is a disorder that makes it hard for your blood to clot. This happens because you don’t have enough of a clotting protein called von Willebrand factor (VWF). It could...
Dec 01, 2009 · Von Willebrand disease is an inherited condition characterized by deficiency of von Willebrand factor, which is essential in hemostasis. The National Heart, Lung, and Blood Institute has released ...
- Barbara P. Yawn, William L. Nichols, Margaret E. Rick
It arises from a deficiency in the quality or quantity of von Willebrand factor (VWF), a multimeric protein that is required for platelet adhesion. It is known to affect several breeds of dogs as well as humans. The three forms of VWD are hereditary, acquired, and pseudo or platelet type.