Acquired von Willebrand disease
Acquired von Willebrand syndrome is a bleeding disorder that is associated with many diseases. Also known as AVWS, this condition involves the dysfunction of the clotting process and abnormal bleeding. Currently, there is no cure for the condition, but there are treatments for controlling the bleeding. This condition is also known as acquired von Willebrand disease.
Definition & Facts
Acquired von Willebrand syndrome (AVWS) is a rare condition characterized by incessant bleeding. and occurs later in one’s life. It results from other ailments. If one is suffering from AVWS, it means they do not have sufficient von Willebrand factor (VWF) in their blood, or it does not work properly.
Von Willebrand Factor (VWF) is a protein found in the blood and is necessary for clotting. Von Willebrand factor carries another substance known as factor VIII that is essential for stimulating the clotting process, and people can have low factor VIII levels. In this case, it is difficult to stop bleeding since the blood cannot clot properly.
This is most commonly the result of genetic mutations, in which case a patient is said to have von Willebrand disease, a genetic disorder. 1 percent of people in the U.S. suffer from von Willebrand disease.
By contrast, if von Willebrand syndrome develops as a result of other conditions it is considered acquired von Willebrand syndrome. Diseases that can cause this condition include multiple myeloma, lung cancer, cardiovascular disease, and non-Hodgkin's lymphoma.
Symptoms & Complaints
- Regular bleeding of the nose and gums
- Dark/black bowel movements
- Red or pink urine
- Heavy periods for women (menorrhagia) or heavy bleeding for those giving birth; menstruation could last a week and one may require double sanitary protection for the bleeding
- Heavy bleeding from wounds, after a tooth is pulled out, or after surgery
- Blood clots of 2.5 centimeters wide
- Bruising accompanied by lumps under one’s skin
Although the cause of AVWD is not always clear, the following are some of the most likely risk factors:
- Diseases: The von Willebrand factor attaches itself to cancerous cells like in the case of kidney tumors, lymphomas, and leukemia. These conditions decrease the levels of von Willebrand factor in the body. Lupus, scleroderma, bone marrow diseases, plasma disorders, and heart disease also increase the risk of getting von Willebrand factor. Hypothyroidism may also decrease von Willebrand factor in the blood. The use of a ventricular assist device also heightens the risk of von Willebrand factor.
- Immune problems: If one has a problem with the immune system, it could cause the body to start attacking its cells. This may cause acquired von Willebrand syndrome.
- Medicines: Medicines that treat depression, infections, or cancer can cause AVWS. Some of the medicines associated with this condition include ciprofloxacin, griseofulvin, and valproic acid.
- Toxic chemicals: If one is regularly exposed to toxic chemicals like pesticides, this increases their chances of getting acquired von Willebrand syndrome.
Diagnosis & Tests
The physician will examine a person’s medical history, conduct a physical examination, and perform diagnostic tests. The doctor will inquire if the patient has the following symptoms or history of symptoms:
- Excessive bleeding when one has undergone a dental procedure or surgery
- Unexpected bruising
- Blood in a patient’s feces
- Bleeding of muscles or joints
- Bleeding after medications like blood thinners, aspirin, and non-steroidal antiinflammatory drugs
- Unexplainable bleeding of the nose that lasts for more than 10 minutes
- Heavy menstrual periods with clots lasting for more than one week
- A history of liver disease, kidney disease, bone marrow disease, or blood disease
A physical exam looks for any bruising or recent signs of bleeding, while blood tests will assess the following:
- Levels of protein or von Willebrand factor antigen
- Factor VIII levels
- How von Willebrand factor is structured
- Platelet function
- The period it takes for the bleeding on a small wound to stop
- Ristocetin cofactor to reveal how VWF works and if it sufficiently clots blood
Treatment & Therapy
Treatment for acquired von Willebrand syndrome is meant to treat the underlying disorder as well as control and prevent bleeding. Common forms of treatment include the following:
- Desmopressin: This medication helps the body synthesize more VWF as well as other elements necessary for the proper clotting of blood.
- Replacement therapy: In this procedure, concentrated VWF is administered using IV infusion
- Corticosteroids: This medication decreases inflammation
- Immunoglobulin: This medicine is administered through IV infusion or a shot to strengthen the immune system.
- Oral contraceptives: Birth control medication with estrogen can help women experiencing heavy periods.
- Antifibrinolytics: Also known as clot stabilizing medications, they delay the breaking down of clotting elements. This keeps the clot intact when it forms especially during dental work or surgery.
- Fibrin sealants: A glue-like substance used directly on wounds to relieve bleeding.
Prevention & Prophylaxis
To minimize the risks of acquired von Willebrand disease, patients are advised to eat a balanced diet and engage in regular exercise. Acquired von Willebrand disease does not interfere with normal activities; however, children are advised to keep away from sporting activities like hockey and football.