We present a 4-year follow-up of a 42-year-old patient with primary thrombocythemia whose clinical course was complicated by two major mucocutaneous bleeding episodes. On both occasions an acquired functional von Willebrand factor deficiency was demonstrated. In contrast to what is reported in the literature, an inverse relationship between platelet number and plasma high-molecular-weight multimers of von Willebrand factor was established.

Acquired von Willebrand disease, Bleeding, Myeloproliferative disorder, Platelets, Primary thrombocythemia, von Willebrand factor
dx.doi.org/10.1007/BF01698487, hdl.handle.net/1765/63297
Annals of Hematology
Department of Hematology

van Genderen, P.J.J, Michiels, J.J, van der Poel-van de Luytgaarde, S.C, & van Vliet, H.H.D.M. (1994). Acquired von Willebrand disease as a cause of recurrent mucocutaneous bleeding in primary thrombocythemia: Relationship with platelet count. Annals of Hematology, 69(2), 81–84. doi:10.1007/BF01698487