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M. Franchini and P.M. Mannucci
AvWS. DDAVP and vWF/FVIII concentrates typically give a prompt but short-lived improvement in vWF and FVIII levels. High doses of vWF/FVIII concentrates usually result in higher response rates, particularly in cases associated with lymphoproliferative disorders, which represent the most challenging condition to treat, and in the context of myeloproliferative neoplasms. In these cases, the short plasma residence time of vWF must be taken into account when dosages and intervals of administration of DDAVP and vWF/FVIII concentrates are planned. Patients with IgG autoantibodies or paraproteins usually respond to HDIVIg with longer-lasting effects. In addition, antifibri- nolytics may be helpful as adjuvant therapy, particularly for the management of mucocutaneous bleeds.
Conclusions
Acquired von Willebrand syndrome is a highly hetero- geneous bleeding disorder, usually characterized by mild to moderate hemorrhagic symptoms that may sometimes be severe, especially when the disease becomes manifest following surgery. Although AvWS is rare, our perception
is that its true incidence is underestimated due to the diag- nostic complexity. When laboratory findings suggest vWD in a patient with a negative personal and family history of a bleeding diathesis, possible AvWS-associated conditions should be explored. We advise screening for AvWS in those patients with conditions potentially associated with this syndrome, particularly onco-hematologic disorders, at the time of the onset of abnormal and otherwise unex- plained bleeding and before undergoing invasive or surgi- cal procedures.
The diagnostic workup of patients with suspected AvWS is challenging. This is mainly due to the overlap in clinical and laboratory features with those of inherited vWD. Diagnosis requires the close integration of hema- tologists with laboratory experts. Similarly, a close inter- action between clinicians from different specialties (i.e. hematology, oncology, rheumatology, cardiology) is needed to appropriately manage patients with AvWS. Besides the control or prevention of bleeding, which often requires the combination of multiple hemostatic medications, the mainstay of treatment is the removal of the underlying conditions. When feasible, this is the only way to cure AvWS.
ing prophylaxis for severe hemophilia A: a population pharmacokinetic model. Haematologica. 2020 Apr 23. doi:
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