From the Guidelines
No, von Willebrand disease is not associated with elevated hematocrit. Von Willebrand disease is a bleeding disorder caused by deficiency or dysfunction of von Willebrand factor, a protein that helps platelets stick to damaged blood vessels and carries clotting factor VIII. This condition typically presents with symptoms of increased bleeding such as easy bruising, nosebleeds, heavy menstrual periods, and prolonged bleeding after injury or surgery. Von Willebrand disease does not affect red blood cell production or concentration, which is what hematocrit measures. Elevated hematocrit (an increased proportion of red blood cells in the blood) is more commonly associated with conditions like polycythemia vera, dehydration, chronic lung disease, or living at high altitude. If a patient with von Willebrand disease has an elevated hematocrit, it would likely be due to a separate, unrelated condition rather than the von Willebrand disease itself, as supported by the guidelines for evaluating and managing von Willebrand disease published by the National Heart, Lung, and Blood Institute (NHLBI) in 2008 1. The classification and laboratory values for von Willebrand disease, as outlined in the guidelines, do not include hematocrit levels, further indicating that von Willebrand disease is not directly associated with elevated hematocrit 1.
Key points to consider in the diagnosis and management of von Willebrand disease include:
- Clinical evaluation strategy to determine which patients would benefit from further diagnostic evaluation for von Willebrand disease or other bleeding disorders 1
- Laboratory values, such as VWF:Rco and VWF:Ag, to diagnose and classify von Willebrand disease 1
- The importance of distinguishing between different types of von Willebrand disease, such as Type 1, Type 2A, Type 2B, Type 2M, Type 2N, and Type 3, based on laboratory values and clinical presentation 1
In clinical practice, it is essential to consider the potential for separate, unrelated conditions that may affect hematocrit levels in patients with von Willebrand disease, and to evaluate and manage these conditions accordingly.
From the Research
Association between von Willebrand's disease and elevated hematocrit (Hct)
- There is no direct evidence in the provided studies to suggest an association between von Willebrand's disease and elevated hematocrit (Hct) 2, 3, 4, 5, 6.
- The studies primarily focus on the diagnosis, treatment, and management of von Willebrand's disease, including its pathophysiology, clinical manifestations, and treatment options 2, 3, 4, 5, 6.
- None of the studies mention hematocrit (Hct) levels or their relationship with von Willebrand's disease 2, 3, 4, 5, 6.
Key findings related to von Willebrand's disease
- Von Willebrand's disease is a common inherited bleeding disorder caused by quantitative or qualitative defects of von Willebrand factor 2, 3, 4, 5, 6.
- The disease is characterized by excessive mucocutaneous bleeding, including nosebleeds, bruising, and bleeding from minor wounds 2, 3, 4, 5, 6.
- Treatment options include desmopressin, von Willebrand factor/factor VIII concentrates, and hemostatic agents 2, 3, 4, 5, 6.