What is Von Willebrand disease (VWD) Normandy type?

From the Guidelines

Von Willebrand disease (VWD) Normandy type is not a recognized or established classification of VWD in the medical literature. Von Willebrand disease is a genetic bleeding disorder caused by deficiency or dysfunction of von Willebrand factor, a protein that helps blood clot 1. It was named after Finnish physician Erik von Willebrand who first described the condition in 1926. The disease affects people worldwide and is not geographically linked to Normandy. Symptoms include easy bruising, nosebleeds, heavy menstrual periods, and prolonged bleeding after injury or surgery.

Classification of VWD

The main types of VWD are as follows:

• Type 1 (partial quantitative deficiency)
• Type 2 (qualitative deficiency) with four subtypes (2A, 2B, 2M, 2N)
• Type 3 (virtually complete quantitative deficiency) 1. Type 1 VWD accounts for ~75% of symptomatic persons, and nearly all the remaining affected persons have Type 2 variants, with Type 2A more common than types 2B, 2M, or 2N. Type 3 VWD is rare, affecting only ~1 person in 1,000.

Diagnosis and Treatment

Treatment typically involves desmopressin (DDAVP) to stimulate release of stored von Willebrand factor, von Willebrand factor concentrates, or other clotting factor replacements depending on the type and severity 1. Some patients may also benefit from antifibrinolytic medications like tranexamic acid to help stabilize blood clots. If you suspect you have a bleeding disorder, it's essential to consult with a hematologist for proper diagnosis and treatment.

Key Points

• VWD is a genetic bleeding disorder caused by deficiency or dysfunction of von Willebrand factor
• The disease affects people worldwide and is not geographically linked to Normandy
• Symptoms include easy bruising, nosebleeds, heavy menstrual periods, and prolonged bleeding after injury or surgery
• Treatment involves desmopressin, von Willebrand factor concentrates, or other clotting factor replacements depending on the type and severity 1

From the Research

Definition and Classification of Von Willebrand Disease

• Von Willebrand disease (VWD) is a genetic bleeding disorder caused by a deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and binds and stabilizes coagulation factor VIII (FVIII) in the blood 2.
• VWD is classified into three types: type 1 (partial deficiency of VWF), type 2 (variable abnormality of VWF structure), and type 3 (severe quantitative deficiency of VWF) 3.

Von Willebrand Disease Normandy Type

• There is no specific mention of "Von Willebrand disease Normandy type" in the provided studies.
• The studies discuss the different types of VWD, including type 1, type 2, and type 3, but do not mention a "Normandy type" 2, 4, 3, 5, 6.

Treatment and Management of VWD

• Treatment strategies for VWD include desmopressin, von Willebrand factor-containing concentrates, and factor VIII concentrates 2, 4, 3, 5.
• Prophylaxis with von Willebrand factor concentrate is considered the standard of care for patients with severe VWD, especially those with type 3 VWD 4, 6.