title: Decreased clearance of von Willebrand factor in a patient with type 2B von Willebrand disease following development of immune thrombocytopenia.
abstract: We report a case of concurrent type 2B von Willebrand disease (VWD) and immune thrombocytopenia (ITP). The patient had characteristic loss of von Willebrand factor (VWF) high molecular weight multimers (HMWM) but a normal platelet count in the initial 8 years after diagnosis of type 2B VWD. When he developed severe thrombocytopenia, however, both his VWD indices and VWF HMWM normalized. As his platelet count increased, he again lost the HMWM and his VWD indices decreased. These results suggest that the severe thrombocytopenia led to decreased clearance of VWF, especially the HMWM.
authors.affiliation: Division of Hematology/Oncology, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan 48201, USA. jwang2@med.wayne.edu
authors.forename: Zhihong J
authors.initials: ZJ
authors.lastname: Wang
authors.forename: Nkechi
authors.initials: N
authors.lastname: Onwuzurike
authors.forename: Michael U
authors.initials: MU
authors.lastname: Callaghan
authors.forename: Madhvi
authors.initials: M
authors.lastname: Rajpurkar
authors.forename: Meera
authors.initials: M
authors.lastname: Chitlur
authors.forename: Jeanne M
authors.initials: JM
authors.lastname: Lusher
mesh_terms: D000293:Adolescent; D019281:Dimerization; D006801:Humans; D008297:Male; D010976:Platelet Count; D013921:Thrombocytopenia / Q000150:complications / Q000175:diagnosis / Q000276:immunology*; D014842:von Willebrand Diseases / Q000150:complications*; D014841:von Willebrand Factor / Q000032:analysis*
chemical_list: D014841:von Willebrand Factor