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Clinical features of children, adolescents, and adults with coexisting hypermobility syndromes and von Willebrand disease
Author(s) -
Hall Sarah A.,
Meier Emily Riehm,
Gupta Sweta,
Nakar Charles,
Rampersad Angeli,
Bakeer Nihal,
Hardesty Brandon,
Kumar Manjusha
Publication year - 2018
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.27370
Subject(s) - medicine , hypermobility (travel) , von willebrand disease , von willebrand factor , disease , pediatrics , dermatology , pathology , physical therapy , platelet
We present five patients with coexistent von Willebrand disease (VWD) and Ehlers‐Danlos syndrome and 21 with VWD and joint hypermobility. Females outnumbered males ten to three, Beighton scores were documented in 58% (15 of 26 patients), and several patients experienced moderately severe bleeding. We believe coexistent hypermobility disorder with VWD potentially affects bleeding severity and want to raise awareness among hematologists. Evaluation by geneticists is recommended because of the varying complexities observed across the disease spectrum, and the availability of new genetic tests should lead to more accurate diagnoses for the various hypermobility disorders.