title: Acquired von Willebrand syndrome in a case of polycythemia vera resulting in recurrent and massive bleeding events in the pleural and abdominal cavity.
abstract: A 52-year-old woman was admitted to the hospital three times in a span of 5 years in hypovolemic shock because of spontaneous and massive bleeding in the pleural and abdominal cavity. Blood tests revealed a high number of blood cells, and bone marrow smears showed trilineage myeloproliferation. Serum erythropoietin level was decreased. Analysis revealed a V617F mutation in the JAK2 protein. Her activated partial thromboplastin time was slightly prolonged, the ratio between von Willebrand factor (vWF) propeptide and vWF antigen was in the normal range, but the ratio between vWF and ristocetin cofactor was decreased dramatically. Further investigation revealed the absence of large and intermediate vWF-multimers. She was diagnosed with polycythemia vera with acquired von Willebrand syndrome. The bleeding was stopped using a transfusion of freshly thawed plasma and cryoprecipitate.
authors.affiliation: aDepartment of Hematology, Qilu Hospital of Shandong University, Jinan bDepartment of Hematology, Affiliated Hospital of Taishan Medcial University cDepartment of Internal Medicine, The Central Hospital of Taian, Taian, China.
authors.forename: Yanchao
authors.initials: Y
authors.lastname: Duan
authors.forename: Jing
authors.initials: J
authors.lastname: Nie
authors.forename: Zhirong
authors.initials: Z
authors.lastname: Zhang
authors.forename: Chunyan
authors.initials: C
authors.lastname: Ji
mesh_terms: D034841:Abdominal Cavity / Q000098:blood supply / Q000473:pathology; D005260:Female; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D008875:Middle Aged; D035422:Pleural Cavity / Q000098:blood supply / Q000473:pathology; D011087:Polycythemia Vera / Q000150:complications*; D014842:von Willebrand Diseases / Q000150:complications*