Von Willebrand disease, type 2B
ICD-10 D68.021 is a billable code used to indicate a diagnosis of von willebrand disease, type 2b.
Von Willebrand disease (VWD) type 2B is a hereditary bleeding disorder characterized by a deficiency or dysfunction of von Willebrand factor (VWF), a protein crucial for platelet adhesion and aggregation. In type 2B, there is a qualitative defect in VWF that leads to increased binding of VWF to platelets, resulting in thrombocytopenia (low platelet count) due to the consumption of platelets in the formation of aggregates. Patients often present with mucosal bleeding, easy bruising, and prolonged bleeding after injury or surgery. The diagnosis is typically confirmed through laboratory tests that assess VWF levels and function, including ristocetin cofactor activity and platelet aggregation studies. Management may involve desmopressin (DDAVP) to increase VWF levels, along with factor replacement therapies in severe cases. Understanding the nuances of this condition is essential for accurate coding and appropriate patient management.
Comprehensive documentation of laboratory findings, family history, and treatment plans.
Patients presenting with unexplained bleeding, easy bruising, or a family history of bleeding disorders.
Ensure accurate differentiation between VWD types and other bleeding disorders.
Genetic testing results and family pedigree charts.
Patients with a family history of bleeding disorders seeking genetic counseling.
Documenting genetic mutations associated with VWD for accurate coding.
Used to assess platelet levels in patients suspected of having VWD.
Document the reason for the CBC and any abnormal findings.
Hematology specialists should ensure comprehensive lab results are included.
The primary treatment for Von Willebrand disease type 2B is desmopressin (DDAVP), which helps to increase the levels of von Willebrand factor in the blood. In severe cases, factor replacement therapy may be necessary.