Chokri Ben Lamine, Adult Hematology and SCT Assistant Consultant at Oncology Center of Excellence at King Faisal Specialist Hospital and Research Center, shared a post on X:
“Type 3 VWD equals near‑total absence of vWF, which is the most severe form.
Family – autosomal recessive; think consanguinity.
Phenotype
Mucocutaneous bleeding (epistaxis, gums, menorrhagia)
Deep bleeding, such as hemarthrosis and muscle bleeding, results in a clinical picture that mimics hemophilia A.
Labs
vWF:Ag and activity absent
Factor VIII very low – less than ten percent.
aPTT prolonged
PT normal
Pearl: DDAVP is ineffective because there is no endogenous von Willebrand factor available to release.
Life-threatening bleeding (ICH, GI, major trauma)