Von Willebrand's disease

Description Von Willebrand dise...

Description

Von Willebrand disease is a bleeding disorder that affects the body's ability to form blood clots. It is most often an inherited disorder that results from low levels of Von Willebrand protein. This protein acts as the glue that holds tiny cells called platelets together during the formation of a clot. Von Willebrand disease is relatively common and affects both males and females.

Symptoms

Symptoms include easy bruising, prolonged bleeding, and significant bleeding during menstrual periods in women (menorrhagia).

Tests

Common tests used for diagnosis and treatment

Workup:
A complete history and physical will be performed. Blood tests will be ordered to evaluate the amount and function of the Von Willebrand factor in your blood.

Tests:
Bleeding time, Complete blood count (CBC), Activated partial thromboplastin (aPTT or PTT), Prothrombin test (PT, INR)

Other Specific Tests: Von Willebrand factor (VWF) antigen, platelet function test (PFA-100), Von Willebrand factor multimers, factor VIII clotting activity, Ristocetin cofactor activity

Specialists:
Internal Medicine, Pediatrics, Family Practice, Blood and Cancer Care (hematology and oncology), Pediatric Hematology and Oncology

Treatment

Treatment with intravenous clotting factors may be needed to stop bleeding. Otherwise, desmopressin (DDAVP) is the most common treatment and can be administered via a nasal spray. DDAVP is a synthetic hormone that stimulates the release of more Von Willebrand factor.