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Von willebrand disease (VWD)

Von willebrand disease (VWD)
Von willebrand disease (VWD)
Von willebrand disease (VWD)
Von willebrand disease (VWD)

Introduction

Von Willebrand Disease is inheritade disease due to deficiency of vWF and patients present with a history of easy bruising, menorrhagea, gum bleeding and spontaneous joint bleeding in severe form, this is a commonest bleeding disorder in the population especially in women.

Risk Factors

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Signs and symptoms

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Diagnostic criteria

• Familial history of bleeding disorder is important
• aptt
• Platelets normal in except in severe form
• Confirmatory test: VWF level assay.

Investigations

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Treatment

  • Non-pharmacological

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  • Pharmacological

    • Tranexamic acid 500mg (PO) 8 hourly until bleeding is stopped.
    OR
    • Etamsylate 500mg (PO) 8 hourly until the bleeding stop

    Note: If there is no response to appropriate replacement therapy tests for inhibitors (an inhibitor is formed when one develops antibodies against factor concentrates) Detection of inhibitor is by aPTT mix study and confirmed by Bethesda assay (BU)

    If no response

    • Desmopresin (DDVAP) infusion 0.3µg/kg IV Max. Dose20µg.
    Note:
    • Patient unresponsive to DDVAP may be treated with virus-inactivated vWF containing FVIII concentrate.
    • Never give Etamsylate or Tranexamic acid to patients bleeding per urethral

Prevention

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Updated on,

14 Novemba 2020 12:33:47

References

    1. STG
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