What is it about?
AVWS is relatively uncommon and an under-diagnosed acquired bleeding disorder that occurs in individuals with no personal or familial history of bleeding diathesis. It is usually associated with an underlying disorder, namely autoimmune, lymphoproliferative, myeloproliferative, malignant, cardiovascular, or other disorders.
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Why is it important?
The diagnosis is challenging because it greatly depends on clinical suspicion. On the other hand, the tests used to assess AVWS are the same as those for VWD, and the differential diagnosis between both can sometimes be difficult. It was possible to observe different laboratory profiles between the patients with lymphoproliferative disor- ders (P1–P3) and myeloproliferative disorders (P4–P6). The multimer analysis was revealed to be the most sensitive tool, confirming the AVWS showed typical patterns according to each pathogenic mechanism.
Perspectives
In our experience, in all individuals with late haemorrhagic episodes and/or AVWS-associated disorders, VWF:Ag, VWF:RCo, VWF:CB and multimer analysis should be performed. This approach enhanced the efficacy of AVWS diagnosis, leading to a more tailored therapeutic management in each patient.
Dr Teresa Fidalgo
Read the Original
This page is a summary of: Acquired von Willebrand syndrome in haematologic malignancies - how the clinical-laboratory correlation improves a challenging diagnosis - a case series, Haemophilia, May 2017, Wiley,
DOI: 10.1111/hae.13243.
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