TREATMENT OF PATIENTS WITH VON WILLEBRAND DISEASE WITH DESMOPRESSIN AND DIAGNOSIS BY MULTIMER ASSAY

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Laboratorinė medicina. 2023,
t. 25,
Nr. 3,
p. 153 -
158

Summary

Introduction. Von Willebrand disease (vWD) is the most common blood clotting disorder, affecting up to 1% of the general population. The diagnosis of this disease is quite difficult and complex. Only a set of various research methods can ensure a reliable diagnosis of vWD, and most importantly, the determination of the type of this disease. One of the most challenging but at the same time opening up opportunities for vWD control is the analysis of Von Willebrand factor (vWF) multimers by electrophoresis. An important aspect is that this test can not only help to differentiate the types of the disease, but also it is useful to monitor desmopressin's response to treatment. The aim of this work is to review the pathophysiology of vWD, diagnosis by electrophoresis, treatment and assessment of response to desmopressin. vWF multimers is carried out only in specialized centers and not in all Baltic countries, thus it becomes extremely important to expand knowledge about this methodology, its advantages and applicability.

Material and methods. Literature review was performed in PubMed, Cochrane Library and Google Scholar databases.

Conclusions. When evaluated together with other vWF tests, multimer analysis allows differentiation of vWD types and/or subtypes – especially 1, 2A/2B, 3. The vWF multimer test is also suitable for monitoring response to desmopressin in patients with type 1 vWD, but so far there are no established recommendations about how long after drug administration blood sample should be taken and how to interpret the results. In order to apply this method to the monitoring of desmopressin response and to ensure objective results, detailed scientific studies are necessary.

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