The hemostasis system in children with hereditary spherocytosis.

Autor: Seregina EA; Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia; Center for Theoretical Problems of Physicochemical Pharmacology RAS, Moscow, Russia. Electronic address: elsereg@inbox.ru., Poletaev AV; Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia., Bondar EV; Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia., Vuimo TA; Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia., Ataullakhanov FI; Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia; Center for Theoretical Problems of Physicochemical Pharmacology RAS, Moscow, Russia; Department of Physics, Moscow State University, Moscow, Russia; The Faculty of Biological and Medical Physics, Moscow Institute of Physics and Technology, Dolgoprudniy, Russia., Smetanina NS; Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia; Pirogov Russian National Research Medical University, Moscow, Russia.
Jazyk: angličtina
Zdroj: Thrombosis research [Thromb Res] 2019 Apr; Vol. 176, pp. 11-17. Date of Electronic Publication: 2019 Feb 05.
DOI: 10.1016/j.thromres.2019.02.004
Abstrakt: Introduction: Patients with hereditary spherocytosis (HS) are characterized by having an increased risk for thrombosis. An early manifestation of thrombotic complications can occur even in childhood, especially after surgery. Hypercoagulability can be associated with hemolytic crises.
Aim: The aim of this study was to investigate the hemostatic state in children with HS using global hemostasis assays.
Methods: The hemostatic status of 62 children (38 boys and 24 girls; age range: 0.5 to 17 years) with HS during and without hemolytic crisis was assessed using clotting times (APTT, TT, and PR), fibrinogen and D-dimer levels, and global hemostasis, thromboelastography (TEG) and thrombodynamics (TD) assays. One hundred and two healthy children undergoing annual medical examination were enrolled as a control group.
Results: TEG and TD parameters were increased in the children with HS compared to the control group (60 ± 5 mm vs. 53 ± 4 mm, p < 0.05 for TEG maximum amplitude; 28 ± 3 μm/min vs. 24 ± 2 μm/min, p < 0.05 for TD clot growth rate), while APTT, TT and PR were not significantly different between the two groups. Patients with HS were divided into 2 groups: those during hemolytic crisis (28 patients) and those without hemolytic crisis (34 patients). TEG and TD parameters were increased in those during hemolytic crisis compared to the steady state HS group (62 ± 5 mm vs. 57 ± 4 mm, p < 0.05 for TEG maximum amplitude; 31 ± 4 μm/min vs. 26 ± 3 μm/min, p < 0.05 for TD clot growth rate). The D-dimer levels were increased in 4 HS patients, for whom the activation of blood clotting was noted. Fibrinogen levels were decreased in patients with HS compared to the control group (2.1 ± 0.4 mg/ml vs. 2.6 ± 0.4 mg/ml, p < 0.05). Other tests were within the reference ranges for both groups.
Conclusions: The global hemostasis tests TEG and TD revealed hypercoagulability in patients with HS. More dramatic changes were observed in patients experiencing a hemolytic crisis.
(Copyright © 2019 Elsevier Ltd. All rights reserved.)
Databáze: MEDLINE
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