In the PALM-COEIN classification (FIGO 2011) for abnormal uterine bleeding, the 'C' in COEIN stands for 'coagulopathy.' Von Willebrand disease (VWD) is the most common systemic coagulation disorder causing heavy menstrual bleeding. The VWD subtype that paradoxically has normal platelet count but requires platelet-directed therapy (DDAVP) rather than factor replacement is:

• A Type 2A VWD (qualitative defect, decreased high-MW multimers)
• B Type 2B VWD (gain-of-function mutation, enhanced platelet binding causing thrombocytopenia)
• C Type 3 VWD (severe quantitative deficiency)
• D Type 1 VWD (quantitative deficiency, mild–moderate) ✓

Explanation

Type 1 VWD (partial quantitative deficiency of VWF) is the most common subtype (~70% of VWD cases) and responds well to DDAVP (desmopressin), which releases endogenous VWF from Weibel-Palade bodies. DDAVP is the treatment of choice for Type 1 VWD with normal platelet count. Type 2B VWD has a paradoxical situation: the mutation causes enhanced binding of VWF to platelets, leading to spontaneous platelet aggregation and thrombocytopenia; DDAVP is CONTRAINDICATED in Type 2B as it worsens thrombocytopenia. Type 2A and 2B require specific VWF concentrate therapy. Type 3 requires factor VIII/VWF concentrate. For menorrhagia, tranexamic acid and oral contraceptives are also effective adjuncts across subtypes.

Reference: Shaw's Textbook of Gynaecology, 17th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.