D68.022
Von Willebrand disease, type 2M
Clinical Classification Guidelines
Inclusion Terms
- Qualitative defects of von Willebrand factor with defective platelet adhesion with a normal size distribution of von Willebrand factor multimers
Medical Intelligence & Overview
Von Willebrand disease (VWD) is a hereditary bleeding disorder caused by problems with a protein called von Willebrand factor (VWF). This protein plays a critical role in blood clotting, helping blood cells stick together to form clots and stop bleeding. Type 2M of VWD, classified under ICD-10 code D68.022, is a specific subtype characterized by qualitative defects in VWF that impair platelet adhesion despite normal levels and multimer sizes of the protein. This detailed guide offers an overview of this condition, covering its causes, symptoms, diagnosis, and potential management strategies.
Causes & Symptoms
Clinical Causes: Inherited genetic mutations affecting the structure or function of von Willebrand factor, especially impacting its ability to mediate platelet adhesion. Mutations that alter the binding sites of VWF necessary for platelet interaction, leading to defective clot formation. Autosomal dominant inheritance pattern, meaning a single copy of the altered gene can cause the disorder. Rare instances of acquired VWF abnormalities due to other medical conditions or medications, though less common in type 2M.
Key Symptoms: Frequent nosebleeds (epistaxis) Easy Bruising: tendency to develop bruises easily from minor injuries Prolonged bleeding after cuts or injuries Heavy menstrual bleeding (menorrhagia) Bleeding from gums or mouth Increased bleeding following surgical procedures or dental work Rare cases of internal bleeding, such as in joints or muscles
Diagnostic & Treatment
Diagnosis Path: Complete blood count (CBC) to evaluate overall blood health and platelet count. Assays measuring the activity and levels of von Willebrand factor (VWF), including VWF antigen tests. VWF activity assays, such as the ristocetin cofactor activity test, to assess functional ability. Multimer analysis to evaluate the size distribution of VWF multimers, which remains normal in type 2M. Platelet function tests to observe how well platelets adhere and aggregate. Genetic testing may be performed for definitive diagnosis, especially in hereditary cases.
Treatment Protocols: Desmopressin (DDAVP): a medication that promotes release of stored VWF from the body's reserves, effective in many mild cases. VWF-containing blood products: administered during significant bleeding or before surgical procedures to provide functional VWF. Antifibrinolytic agents (such as tranexamic acid): used to prevent clot breakdown in mucous membranes, especially during dental procedures. Avoidance of medications that impair platelet function, such as aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs). Regular monitoring and tailored management plans for individuals with frequent or severe bleeding episodes. Genetic counseling for affected families to understand inheritance patterns and risks.
Clinical Advice & FAQs
Billing Guidance
Is D68.022 a billable ICD-10 code?
Yes, D68.022 is a specific, billable code that can be used to indicate a diagnosis for reimbursement purposes.
Documentation
How do I report D68.022?
Clinical documentation must specify the nature of Von Willebrand disease, type 2M and any associated comorbidities for accurate reporting.
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