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Introduction von Willebrand disease ( VWD) is the most common inherited bleeding disorder. The age of bleeding onset is highly variable, also in patients with similar degree of severity. Aim The primary aim of this study was to evaluate whether baseline factor VIII ( FVIII) plasma levels correlate with age at first bleeding in patients with extremely low levels of VWF: RCo (<6 IU dL−1). Methods One hundred and three patients with VWF: RCo <6 IU dL−1 (6 VWD1, 73 VWD2 and 24 VWD3) undergoing a medical examination between September 2010 and September 2013 were included. The relationship between baseline FVIII levels and age at first bleeding was tested in a multivariable linear regression model, adjusting for sex. Results The median age at first bleeding was lower in patients with VWD3 than in those with severe forms of VWD1 or VWD2 (1 year vs. 7 and 8 years, respectively, P < 0.0001). A positive non-linear relationship between FVIII levels and age at first bleeding was found, the latter increasing by 5 years for every 10 IU dL−1 increase of FVIII (β = 4.95 [95% CI: 2.02-7.87]) until levels of 30 IU dL−1, after which the age increased slowly. This relationship was not found in VWD 2A and 2B. In 65 patients (63%) there was a more than 6-month delay between bleeding onset and VWD diagnosis, with no difference over decades. Conclusions Baseline FVIII plasma levels influence the age at bleeding onset in VWD patients with extremely low levels of VWF: RCo, except in those with types 2A and 2B. [ABSTRACT FROM AUTHOR]

The article presents a case study of a woman who was diagnosed having a type 2B von Willebrand disease, eight years following investigations for severe epistaxis. Her bleeding time, factor VIII, and antigen level, Ristocetin cofactor activity, lack of high molecular weight and platelet count were assessed. The patient had an early foetal loss at the age of 37 and underwent revision of uterine cavity. The patient did not show any significant change after treatment due to severe thrombocytopenia.

Patients with von Willebrand disease ( VWD) may need orthopaedic surgery because of disabling chronic arthropathy due to recurrent joint bleeding. They may also require this surgery independently of their haemostasis disorder. Knowledge regarding the management of orthopaedic surgery in VWD is limited. Description of management of orthopaedic surgery in patients with VWD, based upon retrospective data collection and analysis of 32 orthopaedic procedures carried out over a period of 33 years in 23 patients was the aim of this study. Of 32 procedures, six were minor (three hand surgery, one foot surgery, two others) and 26 were major (seven joint replacements, nine arthroscopic procedures, two foot surgery, eight others). Twenty-two procedures were performed using replacement therapy with plasma-derived concentrates containing both factor VIII ( FVIII) and von Willebrand factor ( VWF). Two procedures in patients with acquired von Willebrand syndrome ( AWVS) were performed using FVIII- VWF concentrates associated with intravenous immunoglobulins, or desmopressin plus tranexamic acid. Seven procedures were performed using desmopressin alone and one using intravenous immunoglobulins in AVWS. Bleeding complications occurred in seven procedures (22%). In one patient, an anti- VWF antibody was diagnosed after surgery. Anticoagulant prophylaxis of venous thromboembolism was implemented in four cases only and in two instances there was excessive bleeding. In conclusion, control of surgical haemostasis was achieved in most patients with VWD undergoing orthopaedic surgery. The control of haemostasis combined with an adequate surgical technique and early post-operative rehabilitation are warranted for the successful performance of orthopaedic surgery in VWD, which requires the involvement of specialized haemophilia centres. [ABSTRACT FROM AUTHOR]