Вопросы гематологии/онкологии и иммунопатологии в педиатрии. 2018; 17: 93-96
Редкая форма тромбоцитопатии с нарушением прокоагулянтной активности и секреции томбоцитов
https://doi.org/10.24287/1726-1708-2018-17-1-93-96Аннотация
Список литературы
1. Rao A.K. Inherited Platelet Function Disorders. Overview and Disorders of Granules, Secretion, and Signal Transduction. Hematol Oncol Clin N Am 2013; 27: 585-611.
2. Selle F., et al. Clinical and Laboratory Findings in Patients with -Storage Pool Disease: A Case Series. Seminars in Thrombosis & Hemostasis.
3. Cai H., Mullier F., Frotscher B., Briquel M.E., Toussaint M., Massin F., et al. Usefulness of Flow Cytometric Mepacrine Uptake/Release Combined with CD63 Assay in Diagnosis of Patients with Suspected Platelet Dense Granule Disorder Semin Thromb Hemost 2016; 42: 282-91.
4. Daskalakis M., Colucci G., Keller P., Rochat S., Silzle T., Biasiutti F.D., et al. Decreased Generation of Procoagulant Platelets Detected by Flow Cytometric Analysis in Patients with Bleeding Diathesis. Cytometry Part B (Clinical Cytometry) 00B:00-00. 2014.
5. Rodeghiero F., Tosetto A., Abshire T., Arnold D.M., Coller B., James P., Neunert C., Lillicrap D. ISTH/SSC joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group. ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost 2010; 8: 2063-5.
6. Suntsova E.V., Demina I.M., Ignatova A.A., Ershov N.M., Trubina N.M., Dobrynina J., Serkova I.V., et al. Bleeding tendency and platelet function during treatment with romiplostim in children with severe immune thrombocytopenic purpura. Int J Hematol 2017; 105 (6): 841-8.
7. van Geffen J.P., Swieringa F., Heemskerk J.W.M. Platelets and coagulation in thrombus formation: aberrations in the Scott syndrome. Thrombosis Research 141S2, 2016; S12-S16.
8. Мазуров А.В. Физиология и патология тромбоцитов. - М.: Литтерра, 2011.
Pediatric Hematology/Oncology and Immunopathology. 2018; 17: 93-96
Combined disorder of procoagulant activity and platelet secretion with normal aggregation in a patient with a bleeding tendency
https://doi.org/10.24287/1726-1708-2018-17-1-93-96Abstract
A 54-year-old woman with a bleeding tendency after surgical interventions revealed abnormalities in the functional activity of platelets in the form of decreased phosphatidylserine exposure and the release of dense and α-granules. The morphology of the platelets remained normal, as was the aggregation function in the Born-O'Brien method with activation of ADP, collagen and ristomycin. In the study of plasma hemostasis, there were no abnormalities. The findings indicate that the patient has an unclassified thrombocytopathy.
References
1. Rao A.K. Inherited Platelet Function Disorders. Overview and Disorders of Granules, Secretion, and Signal Transduction. Hematol Oncol Clin N Am 2013; 27: 585-611.
2. Selle F., et al. Clinical and Laboratory Findings in Patients with -Storage Pool Disease: A Case Series. Seminars in Thrombosis & Hemostasis.
3. Cai H., Mullier F., Frotscher B., Briquel M.E., Toussaint M., Massin F., et al. Usefulness of Flow Cytometric Mepacrine Uptake/Release Combined with CD63 Assay in Diagnosis of Patients with Suspected Platelet Dense Granule Disorder Semin Thromb Hemost 2016; 42: 282-91.
4. Daskalakis M., Colucci G., Keller P., Rochat S., Silzle T., Biasiutti F.D., et al. Decreased Generation of Procoagulant Platelets Detected by Flow Cytometric Analysis in Patients with Bleeding Diathesis. Cytometry Part B (Clinical Cytometry) 00B:00-00. 2014.
5. Rodeghiero F., Tosetto A., Abshire T., Arnold D.M., Coller B., James P., Neunert C., Lillicrap D. ISTH/SSC joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group. ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost 2010; 8: 2063-5.
6. Suntsova E.V., Demina I.M., Ignatova A.A., Ershov N.M., Trubina N.M., Dobrynina J., Serkova I.V., et al. Bleeding tendency and platelet function during treatment with romiplostim in children with severe immune thrombocytopenic purpura. Int J Hematol 2017; 105 (6): 841-8.
7. van Geffen J.P., Swieringa F., Heemskerk J.W.M. Platelets and coagulation in thrombus formation: aberrations in the Scott syndrome. Thrombosis Research 141S2, 2016; S12-S16.
8. Mazurov A.V. Fiziologiya i patologiya trombotsitov. - M.: Litterra, 2011.
