Acquired von Willebrand syndrome in MPN

Current understanding

To be populated as evidence accumulates.

2026-10-07 — Aberrant erythrocyte rigidity in JAK2myeloproliferative neoplasms underlies concurrent thrombosis and hemorrhage.: cross-connection with erythrocyte-rigidity-clot-pathology: Both mechanisms independently predict the thrombosis–hemorrhage paradox in MPN; erythrocyte rigidity (platelet/plasma-independent) and AVWS (plasma-factor-dependent) may be complementary substrates for concurrent occlusion and bleeding. (novelty: 5)
2026-07-17 — Philadelphia-negative myeloproliferative neoplasms and acquired von Willebrand syndrome: only pertinent at diagnosis?: Single-center cohort found AVWS (VWF:GPIbM/VWF:Ag <0.65) in >1/3 of newly diagnosed PV/ET/prefibrotic MF, independently associated with female sex, platelets >700×10⁹/L, and polycythemia — reframing AVWS as a marker of overall proliferative burden rather than extreme thrombocytosis alone; correlated with prior bleeding but not long-term bleeding/thrombosis-free survival, and nearly always corrected on follow-up. (novelty: 3)