FEATURES OF DIAGNOSTICS OF BERNARD-SOULIER SYNDROME

M. A. Kumskova; O. Yastrubinetskayc; E. A. Likhachyova; S. A. Vasiliev; V. N. Dvirnyk; N. Konyashina; L. S. Al-Radi; T. N. Moiseeva; N. Zozulya

doi:10.18821/0234-5730-2016-61-4-217-221

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FEATURES OF DIAGNOSTICS OF BERNARD-SOULIER SYNDROME

Journal article

Peer reviewed

FEATURES OF DIAGNOSTICS OF BERNARD-SOULIER SYNDROME

M. A. Kumskova, O. Yastrubinetskayc, E. A. Likhachyova, S. A. Vasiliev, V. N. Dvirnyk, N. Konyashina, L. S. Al-Radi, T. N. Moiseeva and N. Zozulya

Gematologiia i transfuziologiia, Vol.61(4), pp.217-221

10/01/2016

DOI: 10.18821/0234-5730-2016-61-4-217-221

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Abstract

Bernard Soulier syndrome (BSS) is the rare platelet disorder. It is caused by the deficiency or defect of the glycoprotein (GP) lb-IX-V complex on the surface of platelets - the main receptor for von Willebrand factor (vWF). Binding of vWF with GP lb-IX-V complex initiates primary hemostasis and provides platelets adhesion at the site of the injured blood vessel. Dissociation of plasma, vascular and thrombocytic hemostasis gives rise to the development of the microcirculatory or mixed hemorrhagic syndrome. The typical features of such types are considered in the clinical case of the BSS female patient. Diagnostic algorithm for BSS includes the evaluation of functional properties, morphology and biochemical characteristics of platelets with the compulsory use of flow cytometry, allowing to reveal the deficiency of GP lb-IX-V complex on the surface of platelets.

Hematology

Life Sciences & Biomedicine

Science & Technology

Details

Title: Subtitle: FEATURES OF DIAGNOSTICS OF BERNARD-SOULIER SYNDROME
Creators: M. A. Kumskova - Natl Res Ctr Hematol, Moscow 125167, Russia
O. Yastrubinetskayc - Natl Res Ctr Hematol, Moscow 125167, Russia
E. A. Likhachyova - Natl Res Ctr Hematol, Moscow 125167, Russia
S. A. Vasiliev - Natl Res Ctr Hematol, Moscow 125167, Russia
V. N. Dvirnyk - Natl Res Ctr Hematol, Moscow 125167, Russia
N. Konyashina - Natl Res Ctr Hematol, Moscow 125167, Russia
L. S. Al-Radi - Natl Res Ctr Hematol, Moscow 125167, Russia
T. N. Moiseeva - Natl Res Ctr Hematol, Moscow 125167, Russia
N. Zozulya - Natl Res Ctr Hematol, Moscow 125167, Russia
Resource Type: Journal article
Publication Details: Gematologiia i transfuziologiia, Vol.61(4), pp.217-221
DOI: 10.18821/0234-5730-2016-61-4-217-221
ISSN: 0234-5730
Publisher: Ministerstvo Zdravookhraneniya
Number of pages: 5
Language: Russian
Date published: 10/01/2016
Academic Unit: Internal Medicine
Record Identifier: 9985121619002771

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