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A subtype of Type 2 characterized by decreased VWF-dependent platelet adhesion. Clinical features include mucocutaneous bleeding and reduced VWF multimer size. Diagnosis is by the absence of high-molecular-weight VWF multimers. Treatment may involve desmopressin and VWF concentrates.

A subtype of Type 2, characterized by increased affinity of VWF for platelets leading to thrombocytopenia. Clinical features include mild to moderate bleeding. Diagnosis by abnormal ristocetin-induced platelet agglutination. Treatment avoids desmopressin as it can worsen thrombocytopenia, and may use VWF concentrates.

A subtype of Type 2, resembling hemophilia A, caused by a defect in the VWF factor VIII binding site. Clinical features include bleeding episodes typical of factor VIII deficiency. Diagnosis involves assays to measure the binding of VWF to factor VIII. Treatment includes VWF concentrates, sometimes combined with factor VIII concentrates.

The most severe form with virtually absent VWF. Clinical features include severe bleeding episodes, often from an early age. Diagnosis is by undetectable levels of VWF antigen and activity, and low factor VIII activity. Treatment includes VWF concentrates and factor VIII replacement therapy.

The most common and mildest form, reduced levels of VWF. Clinical features include mild to moderate bleeding, such as nosebleeds, gum bleeding, and menorrhagia. Diagnosis is by low VWF antigen and activity levels. Treatment often involves desmopressin (DDAVP) to release stored VWF.

Characterized by dysfunctional VWF, has four subtypes (2A, 2B, 2M, 2N). Clinical features depend on subtype but often involve abnormal bleeding. Diagnosis typically includes specialized tests like ristocetin cofactor assay. Treatment may involve VWF concentrates that possess the functional properties lacking in patients.