Hematology Clinical Resource Area: Von Willebrand Disease Overview

This hematological disorder is of genetic inheritance. Those born with the disease have ineffective or missing von Willebrand proteins in the blood. This is a clotting disorder. There are three types of von Willebrand Disease; Type 1, Type 2 or Type 3. Type 1 is related to a low level of the protein. Type 2 is related to ineffective functioning of the protein. Type 3 is related to absence of the protein. Type 1 and 2 are a dominant gene disorder and Type 3 is recessive.

This disease is treated by the symptoms. The patient will require transfusion support of RBC and chelating therapy as well. The patient with Type 1 or 2 may receive a synthetic hormone Desmopressin as a nasal spray or injection. Oral contraceptives are sometimes given to females patients who experience heavy menstrual periods with excessive bleeding.

Diagnosis: This disease is difficult to diagnose. Diagnosis requires a skillful practitioner obtaining a thorough patient and family history in addition to physical assessment. Patients will present with bleeding symptoms; heavy bleeding, nosebleeds, bruising, blood to excrements, or excessive bleeding with trauma. The disease is diagnosed by assessing for anemia. In addition the patient will be assessed for von Willebrand protein and how well it functions. The practitioner may obtain a factor VIII clotting test as well. Practitioners may also order a platelet function test to assess how well the platelets are clotting.

Disease Overview: Patients should be advised to avoid aspirin, Ibuprofen and platelet altering medications. This is a lifelong disease and will require frequent laboratory testing, practitioner assessment and compliance with therapy. Patient and caregivers should be alerted to need to inform all health care providers of von Willebrand diagnosis. Patient should be cautioned regarding need for close monitoring and assessment prior to any invasive procedure, dental procedures or trauma. Exercise is necessary but patients should avoid contact or traumatic events.

Additional Resources: National Heart Lung and Blood Institute; Diseases and Conditions Index
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