Acquired von Willebrand Syndrome

Acquired von Willebrand syndrome in haematologic malignancies – how the clinical‐laboratory correlation improves a challenging diagnosis – a case series

“…Likewise, the multimer analysis was revealed to be the most sensitive tool, confirming the AVWS showed typical patterns according to each pathogenic mechanism. These findings agreed with other previously described studies [1,2,6].…”

“…The common laboratory features of these three patients are characterized by thrombocythemia in which the adsorption of large VWF multimers was demonstrated on activated platelets with an inverse relationship between the platelet counts and the presence of HMWM in the plasma. Therefore, the proteolysis of HMWM may be triggered by plateletreleased calcium ions, causing the activation of proteases and elastases [1,2,6]. However, because of the high correlation of platelets/multimers, it is reasonable to assume that additional risk factors, such as platelet function defects, could contribute to bleeding in these patients [6].…”

“…This case serves as an important example of successful management of non-responsive urinary tract bleeding to bypassing agent with rpFVIII and subsequent inhibitor eradication. 1 [1][2][3][4][5]. A variety of pathogenic mechanisms have been proposed: (i) the presence of specific or non-specific autoantibodies that form circulating immune complexes, (ii) the adsorption of von Willebrand factor (VWF) onto neoplastic cell clones, and (iii) the loss of high-molecular-weight multimers (HMWM) after exposure to high shear stress or proteolysis.…”

Advantage of recombinant von Willebrand factor for peri‐operative management in paediatric acquired von Willebrand syndrome

“…The efficacy of DDAVP is likely to be limited, and patients with cardiovascular disease have been reported to have the worst outcomes with its use with therapeutic responses only seen in 10%. 5 Additionally, DDAVP provided additional obstacles in our patient given the haemostatic challenges inherent to neurosurgery, his young age and risk of hyponatremia, and concern for further elevation of his FVIII.…”

Pregnancy in patient with hereditary hemorrhagic telangietasia (osler-weber-rendu disease) and pulmonary arteriovenous malformations: case report

“…1−4 The term of aVWs is now accepted for patients with predominant underlining diseases that lead to defective von Willebrand factor (VWF). 5 In recent years there has been a progressive increase of publications concerning patients with defective VWF in whom the family history and the late onset of bleeding symptoms speak against an inherited form. 6,7 We report the case of a woman who developed severe dyspnoe, cough, haemoptysis during 34-week of pregnancy.…”