Acquired von Willebrand syndrome: an underdiagnosed and misdiagnosed bleeding complication in patients with lymphoproliferative and myeloproliferative disorders.
Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder with laboratory findings similar to those for congenital von Willebrand disease (VWD). Unlike the congenital disease, AVWS usually occurs in individuals with no personal or family history of bleeding. The prevalence of AVWS in the general population is unknown because data from large prospective studies of this syndrome are not available. Although AVWS is particularly frequent in lymphoproliferative or myeloproliferative disorders, it can also be associated with solid tumors, immunologic and cardiovascular disorders, and other miscellaneous conditions. Diagnosis of AVWS is based on assays measuring the activity of von Willebrand factor (VWF). This tends to be abnormally low, but factor VIII (FVIII) coagulant activity can sometimes be normal. FVIII/VWF inhibiting activity is found in only a minority of cases. Bleeding episodes in patients with AVWS are mostly of the mucocutaneous type and can be managed with desmopressin, plasma-derived FVIII/VWF concentrates, and intravenous immunoglobulin (IVIg). Recombinant activated factor VII can be useful in patients unresponsive to standard therapy. An updated version of the International Registry on AVWS, recently available online, will provide more information on this rare, but underdiagnosed and misdiagnosed, disorder.
Federici
Augusto B
AB
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Department of Internal Medicine and Dermatology, IRCCS Foundation Maggiore Policlinico Hospital, Mangiagalli, Italy. augusto.federici@unimi.it
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000941:Antigens / Q000378:metabolism / Q000627:therapeutic use; D001777:Blood Coagulation; D001780:Blood Coagulation Tests; D002318:Cardiovascular Diseases / Q000150:complications / Q000175:diagnosis / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D020147:Coagulation Protein Disorders / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism / Q000628:therapy; D003951:Diagnostic Errors; D005167:Factor VII / Q000627:therapeutic use; D005169:Factor VIII / Q000378:metabolism / Q000627:therapeutic use; D015942:Factor VIIa; D005260:Female; D006470:Hemorrhage / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism / Q000628:therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000175:diagnosis / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000150:complications / Q000175:diagnosis / Q000378:metabolism; D009369:Neoplasms / Q000150:complications / Q000175:diagnosis / Q000378:metabolism; D011994:Recombinant Proteins / Q000627:therapeutic use; D013577:Syndrome; D014841:von Willebrand Factor / Q000276:immunology
D000941:Antigens; D016756:Immunoglobulins, Intravenous; D011994:Recombinant Proteins; C029417:Von Willebrand antigen; D014841:von Willebrand Factor; C078147:F8 protein, human; D005167:Factor VII; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa