A case of acquired von Willebrand's disease (AvWD) associated with an adrenal cortical carcinoma is reported. The circulating highest molecular weight multimers (HMWM) of von Willebrand factor (vWF) were decreased when assessed by SDS-agarose plasma electrophoresis, leading to the diagnosis of type II AvWD. No forms of inhibitor could be detected in the plasma of the patient. In contrast, indirect immunoperoxidase studies with a monoclonal antibody to vWF demonstrated an absorption of vWF into malignant cells. Infusion of a vWF-FVIII concentrate, containing significant amounts of HMWM of vWF, allowed surgical resection of the tumour. After the first infusion of the concentrate, the vWF-RCo recovery was found to be low (38%) compared to the vWF:Ag (75%) and FVIII:C (163%) recoveries. The resolution of all biological signs of vWD, including the abnormal multimeric pattern, in the post-operative period was prompt and permanent. Therefore, the absorption of the HMWM of vWF by carcinomatous cells appears to represent a likely pathophysiological mechanism responsible for the AvWD syndrome in this patient.

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The reduction in mortality specifically from breast cancer, demonstrated in the major meta-analyses in the 1980s resulted in public health breast cancer screening programs being set up in many countries, including France. Recent publications have challenged the usefulness of screening, by insisting in particular on the negative effects of overdiagnosis and the lack of any significant impact on mortality. From analysis of the literature and particularly independent reviews published in 2012, we provide some answers for doctors faced with the legitimate concerns of women. These studies confirm that screening in the right age group reduces specific mortality by at least 20% at a cost of overdiagnosis estimated at between 1 and 15%.