Email this article Modern approach to diagnosis of inherited functional platelet disorders
- Authors: Fedorova D.V.1, Zharkov P.A.1, Plyasunova S.A.1, Seregina E.A.1, Ignatova A.A.1
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Affiliations:
- National Scientific and Practical Centre of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev
- Issue: Vol 16, No 1 (2017)
- Pages: 83-95
- Section: Статьи
- Submitted: 10.08.2018
- Published: 10.02.2017
- URL: https://hemoncim.com/jour/article/view/84
- DOI: https://doi.org/10.24287/1726-1708-2017-16-1-83-95
- ID: 84
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Abstract
Inherited functional platelet disorders (IFPD) are very heterogeneous group of hemorrhagic diseases that are difficult to diagnose. Prevalence of IFPD in general population is probably underestimated. In most cases IFPD cause mild to moderate bleeding symptoms in everyday life but may result in life-threating bleeding after trauma and surgical interventions. Rational diagnostic approach is critical to prevent these complications. Data on diagnostic algorithms as well as on benefits and disadvantages of specific laboratory methods are presented in this review. Approach to clinical evaluation is also reviewed with particular attention to bleeding assessment tools. The main emphasis is made on light transmission aggregometry, measurement of platelet granules content and release, flow cytometry. Problems of standardization of platelet functional tests are outlined. Diagnostic algorithms are applied according to the up to date recommendations of International Society on Thrombosis and Haemostasis.
About the authors
D. V. Fedorova
National Scientific and Practical Centre of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev
Author for correspondence.
Email: darya.v.fedorova@gmail.com
MD, pediatrician of Outpatient Consultative Unit
Russia 117997, Moscow, Samory Mashela st., 1
+7 (495) 287-6570, ext. 1702
Russian FederationP. A. Zharkov
National Scientific and Practical Centre of Pediatric Hematology, Oncology and Immunology named after Dmitry RogachevRussian Federation
S. A. Plyasunova
National Scientific and Practical Centre of Pediatric Hematology, Oncology and Immunology named after Dmitry RogachevRussian Federation
E. A. Seregina
National Scientific and Practical Centre of Pediatric Hematology, Oncology and Immunology named after Dmitry RogachevRussian Federation
A. A. Ignatova
National Scientific and Practical Centre of Pediatric Hematology, Oncology and Immunology named after Dmitry RogachevRussian Federation
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