The commonest inherited bleeding disorder worldwide is characterized by reduced plasma von Willebrand factor, autosomal dominant inheritance, proportionate reduction of factor VIII, and a normal platelet count. It responds to desmopressin. This describes which subtype?

• A Type 3 von Willebrand disease
• B Type 2A von Willebrand disease
• C Type 2N von Willebrand disease
• D Type 1 von Willebrand disease ✓

Explanation

Type 1 vWD is a quantitative partial deficiency of normally functioning von Willebrand factor, accounting for about 70 percent of cases, with autosomal dominant inheritance and good response to desmopressin, which releases stored vWF from endothelial Weibel-Palade bodies. Type 3 is a near-total quantitative deficiency, usually autosomal recessive, and type 2 variants are qualitative defects.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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