Abstract Number: PB0949
Meeting: ISTH 2021 Congress
Theme: Platelet Disorders, von Willebrand Disease and Thrombotic Microangiopathies » VWF and von Willebrand Factor Disorders - Clinical Conditions
Background: The diagnostic capabilities of detecting von Willebrand disease(VWD) in children are limited in many regions of our country, and therefore, in 2019, the Russian Hemophilia Society starts the centralized diagnostics program(Program) that provides for distance diagnosis of VWD in the regions.
Aims: Evaluate the first results of centralized program for VWD diagnostics in children.
Methods: According to clinical state of the patient and to the decision of hematologist the patient was enrolled in Program. Blood samples were collected from peripheral vessel, then freezed and transported to the Laboratory of hemostasis at the Dmitry Rogachev National Medical Research Center Of Pediatric Hematology, Oncology and Immunology. There were 122 frozen samples from 122 children under the age of 18 year, from 21 regions of Russian Federation, received during the period from 04.2019 to 02.2021. The vWF:Ag and vWF:RCo and fVIII activity were evaluated in samples.
Results: Preanalytical errors were detected in 5(4.1%)samples. The decrease in vWf activity within 30-50% was detected in 22% of cases, vWF:RCo less than 30% in 13.9% of cases. The vWF:Ag was within 30-50% in 16.4% and less than 30% in 6.5% of samples respectively. Among patients with vWf:RCo <30% type 1 vWD was detected in 4(23.5%)patients, type 2 – in 12(70.6%)patients, type 3 – in 1(5.9%)patient. The decrease in fVIII activity while vWF:Ag and activity were in normal range was observed in 6(4.9%)cases. The decrease in fVIII/vWF:Ag ratio was detected in 8(6.6%)children. The centralized diagnostics program allowed to reveal the hemostasis pathology in 36(29.5%)patients. The 13,9% of all cases had the typical laboratory pattern of vWD, and 20(16.3%)patients require the additional diagnostics using an expanded panel of laboratory tests.
Conclusions: This program has great potential, especially in sparsely populated regions of the country, where the establishment of regional diagnostics on site may be economically impractical.
To cite this abstract in AMA style:
Poletaev A, Seregina E, Fedorova D, Zharkov P. Modern Diagnosis of von Willebrand Disease in Children in Russia: Experience in Centralized Diagnostics [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/modern-diagnosis-of-von-willebrand-disease-in-children-in-russia-experience-in-centralized-diagnostics/. Accessed November 27, 2021.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/modern-diagnosis-of-von-willebrand-disease-in-children-in-russia-experience-in-centralized-diagnostics/