Email this article Diagnostics of inherited functional platelet disorders (IFPD) in children: correlations between results of flow cytometry, clinical phenotype and results of light transmission aggregometry
- Authors: Fedorova D.V.1, Zharkov P.A.1, Ignatova A.A.1, Fedotov A.Y.1, Polokhov D.M.1, Poletaev A.V.1, Seregina E.A.1, Pshonkin A.V.2
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Affiliations:
- Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
- Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Department of Hematology, Oncology and radiotherapy Russian National Research Medical University named after N.I. Pirogov
- Issue: Vol 17, No 1 (2018)
- Pages: 16-22
- Section: Статьи
- Submitted: 09.08.2018
- Published: 09.02.2018
- URL: https://hemoncim.com/jour/article/view/22
- DOI: https://doi.org/10.24287/1726-1708-2018-17-1-16-22
- ID: 22
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Abstract
Inherited functional platelet disorders (IFPD) are very heterogeneous group of hemorrhagic diseases. Light transmission aggregometry (LTA) and flow cytometry of platelets are used for diagnostics of IFPD. Flow cytometry is the newer method with some advantages. Our aim was to access correlation between results of LTA and platelet flow cytometry, as well as relationship between results of flow cytometry and clinical phenotypes. Fifty children with suspicion of IFPD were included in initial retrospective analysis. Patients with thrombocytopenia were excluded. Severity of clinical phenotype was accessed by means of ISTH BAT score. Seven patients were diagnosed as having Glanzmann thrombasthenia (GT). Deficiency of δ- and/or α-granules was discovered in 13 children, deficiency of active form of integrin αIIbβ3 in 5, and its combination in 4. None of patients with granules deficiency had pathological findings in LTA results. ISTH BAT score was significantly higher in children with GT in comparison with other patients. But difference in ISTH BAT score was absent between groups with other defects and without any pathological findings in laboratory tests. Our results correspond with previously published data on low sensivity of LTA towards platelet granule disorders. Use of ISTH BAT score was unable to distinguish patients with functional platelet defects and those without any defects accordingly flow cytometry results.
About the authors
D. V. Fedorova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
Author for correspondence.
Email: daria.fedorova@fcchomoscow.ru
ORCID iD: 0000-0003-4567-1871
MD, hemotologist of Outpatient Consultative Unit
Russia 117997, Moscow, Samory Mashela st., 1
+7 (495) 287-6570, ext. 1702
P. A. Zharkov
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0003-4384-6754
Russian Federation
A. A. Ignatova
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0002-5217-3937
Russian Federation
A. Yu. Fedotov
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyRussian Federation
D. M. Polokhov
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyRussian Federation
A. V. Poletaev
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology
ORCID iD: 0000-0001-5209-2099
Russian Federation
E. A. Seregina
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyRussian Federation
A. V. Pshonkin
Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, ImmunologyDepartment of Hematology, Oncology and radiotherapy Russian National Research Medical University named after N.I. Pirogov
Russian Federation
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