perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2024-69-2-72-77perinatology-1973Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESРезультаты лечения пациентов с впервые диагностированной иммунной тромбоцитопенией: оправдано ли следование клиническим рекомендациям?Results of treatment of patients with newly diagnosed immune thrombocytopenia: is it justified to follow clinical recommendations?https://orcid.org/0000-0001-9777-1220ДолгополовИ. С.DolgopolovI. S.

Долгополов Игорь Станиславович - д.м.н., зам. гл. врача по лечебной работе; зав. кафедрой педиатрии педиатрического факультета

170100 Тверь, наб. Степана Разина, д. 23

Tver

https://orcid.org/0009-0001-4067-2130МнацаканянА. М.MnatsakanianA. M.

Мнацаканян Арсен Мхитарович - врач–детский онколог

170100 Тверь, наб. Степана Разина, д. 23

Tver

https://orcid.org/0009-0004-9866-1823ИвановаА. В.IvanovaA. V.

Иванова Анна Валерьевна - зав. отделением педиатрии Детской областной клинической больницы, асс. кафедры педиатрии педиатрического факультета

170100 Тверь, наб. Степана Разина, д. 23

Tver

https://orcid.org/0009-0004-1390-2529ВолянскаяА. Д.VolianskayaA. D.

Волянская Анастасия Денисовна - студент VI курса педиатрического факультета

170100, г. Тверь, Советская, д. 4

Tver

https://orcid.org/0009-0004-1390-2529НаходноваЕ. А.NakhodnovaE. A.

Находнова Елена Андреевна - студент VI курса педиатрического факультета

170100, г. Тверь, Советская, д. 4

Tver

https://orcid.org/0000-0002-8398-7001РыковМ. Ю.RykovM. YuU.

Рыков Максим Юрьевич - д.м.н., доц., зав. кафедрой педиатрии

129226 Москва, ул. Вильгельма Пика, 4, стр. 1

Moscow

wordex2006@rambler.ruhttps://orcid.org/0000-0003-3372-9925ЗайцеваА. В.ZaitsevaA. V.

Зайцева Анна Васильевна - гл. врач

170100 Тверь, наб. Степана Разина, д. 23

Tver

ГБУЗ Тверской области «Детская областная клиническая больница»; ФГБОУ ВО «Тверской государственный медицинский университет» Минздрава РоссииРоссияChildren’s Regional Clinical Hospital; Tver State Medical UniversityRussian FederationГБУЗ Тверской области «Детская областная клиническая больница»РоссияChildren’s Regional Clinical HospitalRussian FederationФГБОУ ВО «Российский государственный социальный университет»РоссияRussian State Social UniversityRussian Federation2024100520246927277Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20242024Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/1973

Иммунная тромбоцитопения — приобретенное иммуноопосредованное заболевание, характеризующееся изолированной транзиторной или персистирующей тромбоцитопенией <100·109/л. Заболеваемость иммунной тромбоцитопенией составляет 4–6,4 на 100 тыс. детей в год.Цель исследования. Анализ результатов лечения впервые диагностированной иммунной тромбоцитопении согласно клиническим рекомендациям ID699, опубликованным на сайте Минздрава России.Материалы и методы. В анализ включены 13 пациентов (46% девочек, 54% мальчиков, средний возраст 9,5 (4–17) года) с иммунной тромбоцитопенией, поступивших в ДОКБ Тверской области в 2023 г. Инфекция предшествовала в 9 (69%) случаях, а вакцинация против кори — в 1(8%). Средний период от момента начала инфекции 11 (5–15) дней. Степень кровотечения: I — 4 (31%), II — 3 (23%), III — 6 (46%). Гематурия наблюдалась в 3 (23%), меноррагия — в 1 (8%) случаях. Средний уровень тромбоцитов на момент поступления составил 9,0 (1,0–86) · 109/л.Результаты. Заболеваемость составила 5,7 на 100 тыс. детского населения. В качестве дебюта терапии дексаметазон в дозе 20 мг/м2, дни 1–3, использовался в 54% случаев; внутривенный иммуноглобулин 1000 мг/кг, день 1 — в 15%, преднизолон 2 мг/кг, 21 день — в 8% и в 23% случаев осуществлялось динамическое наблюдение. В 2 (17%) случаях потребовались прекращение терапии стероидами и переход на внутривенный иммуноглобулин в связи с нарастанием геморрагического синдрома и/или развитием осложнений терапии. Частичный и полный ответы достигнуты в 8 (62%) и 4 (31%) случаях соответственно. В 1 (8%) случае ответ не мог быть оценен. Суммарная эффективность терапии первой линии составила 92%.Заключение. Применение клинических рекомендаций ID699 показало высокую эффективность для достижения первичного ответа и предупреждения рецидивов иммунной тромбоцитопении у детей.

Immune thrombocytopenia (ITP) is an acquired immune-mediated disease characterized by isolated transient or persistent thrombocytopenia < 100·109/L. The incidence of immune thrombocytopenia is 4–6,4 per 105 children/year.Purpose. To analyze the results of treatment newly diagnosed immune thrombocytopenia according to the clinical guidelines (ID699) published on the website of the Ministry of Health of Russia.Material and methods. The analysis included 13 patients (F–46%, M–54%, median age — 9.5 (4–17) years) with immune thrombocytopenia admitted to the Tver Regional children hospital in 2023. A history of infection preceding the immune thrombocytopenia was in 69% of cases and measles vaccination in 8%. The median period from the onset of infection was 11 (5–15) days. Degree of bleeding was — 1 gr. — 4 (31%), 2 gr. — 3 (23%), 3 gr. — 6 (46%). Hematuria was observed in 3 (23%), menorrhagia in 1 (8%) case. The mean platelet count at the time of admission was 9,0 (1.0–86) · 109/l.Results. The incidence was 5.7 per 105 children/year. Dexamethasone 20 mg/m2, days 1–3, was used in 54% of cases; IVIG 1000 mg/kg, day 1 in 15%, prednisolone 2 mg/kg, day 21 in 8% and in 23% of cases dynamic follow-up was performed. In 2 cases (17%), discontinuation of steroid therapy and switching to IVIG was required due to a hemorrhagic syndrome deterioration or/and complication of steroid therapy. Partial and complete responses were achieved in 8 (62%) and 4 (31%) cases respectively. In 1 (8%) case, the response could not be evaluated. The summary efficacy of first-line therapy was 92%.Conclusion. The clinical guidelines ID699 was highly effective in achieving a primary response and preventing recurrence of immune thrombocytopenia in children.

детииммунная тромбоцитопенияклинические рекомендациилечениеchildrenimmune thrombocytopeniapractice guidelinefirst-line therapyReferencesRodeghiero F., Stasi R., Gernsheimer T., Michel M., Provan D., Arnold D. et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: Report from an international working group. Blood 2009; 113(11): 2386–2393. DOI: 10.1182/blood-2008–07–162503Rodeghiero F., Stasi R., Gernsheimer T., Michel M., Provan D., Arnold D. et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: Report from an international working group. Blood 2009; 113(11): 2386–2393. DOI: 10.1182/blood-2008–07–162503Consolini R., Legitimo A., Caparello M.C. The Centenary of Immune Thrombocytopenia — Part 1: Revising Nomenclature and Pathogenesis. Front Pediatr 2016; 4: 102. DOI: 10.3389/fped.2016.00102Consolini R., Legitimo A., Caparello M.C. The Centenary of Immune Thrombocytopenia — Part 1: Revising Nomenclature and Pathogenesis. Front Pediatr 2016; 4: 102. DOI: 10.3389/fped.2016.00102Provan D., Semple J.W. Recent advances in the mechanisms and treatment of immune thrombocytopenia. EBioMedicine 2022; 76: 103820. DOI: 10.1016/j.ebiom.2022.103820Provan D., Semple J.W. Recent advances in the mechanisms and treatment of immune thrombocytopenia. EBioMedicine 2022; 76: 103820. DOI: 10.1016/j.ebiom.2022.103820Министерство Здравоохранения Российской Федерации, Клинические рекомендации. Иммунная тромбоцитопения. ID699, 2021–2023 гг.Ministry of Health of the Russian Federation, Clinical guidelines. Immune thrombocytopenia. ID699, 2021–2023. (in Russ.) https:// cr.minzdrav.gov.ru/schema/699_1 / Ссылка активна на 25.12.2023.Schoonen W.M., Kucera G., Coalson J., Li L., Rutstein M., Mowat F. et al. Epidemiology of immune thrombocytopenic purpura in the General Practice Research Database. Br J Haematol 2009; 145(2): 235–244. DOI: 10.1111/j.1365–2141.2009.07615.xSchoonen W.M., Kucera G., Coalson J., Li L., Rutstein M., Mowat F. et al. Epidemiology of immune thrombocytopenic purpura in the General Practice Research Database. Br J Haematol 2009; 145(2): 235–244. DOI: 10.1111/j.1365–2141.2009.07615.xProvan D., Arnold D.M., Bussel J.B., Chong B.H., Cooper N., Gernsheimer T. et al. Updated international consensus report on the investigation and management of primary immune thrombocytopenia. Blood Adv 2019; 3: 3780–3817. DOI: 10.1182/bloodadvances.2019000812Provan D., Arnold D.M., Bussel J.B., Chong B.H., Cooper N., Gernsheimer T. et al. Updated international consensus report on the investigation and management of primary immune thrombocytopenia. Blood Adv 2019; 3: 3780–3817. DOI: 10.1182/bloodadvances.2019000812Heitink-Pollé K.M.J., Uiterwaal C.S.P.M., Porcelijn L., Tamminga R.Y.J., Smiers F.J., Van Woerden N.L. et al. Intrave-thrombocytopenia: A randomized controlled trial. Blood 2018; 132: 883–891. DOI: 10.1182/blood-2018–02–830844Heitink-Pollé K.M.J., Uiterwaal C.S.P.M., Porcelijn L., Tamminga R.Y.J., Smiers F.J., Van Woerden N.L. et al. Intrave-thrombocytopenia: A randomized controlled trial. Blood 2018; 132: 883–891. DOI: 10.1182/blood-2018–02–830844Постановление Правительства Российской Федерации от 17.11.2021 № 1968 «Об утверждении Правил поэтапного перехода медицинских организаций к оказанию медицинской помощи на основе клинических рекомендаций, разработанных и утвержденных в соответствии с частями 3, 4, 6–9 и 11 статьи 37 Федерального закона «Об основах охраны здоровья граждан в Российской Федерации»Decree of the Government of the Russian Federation of November 17, 2021 No. 1968 "On approval of the Rules for the phased transition of medical organizations to the provision of medical care based on clinical recommendations developed and approved in accordance with parts 3, 4, 6–9 and 11 of Article 37 of the Federal Law" On the basics of protecting the health of citizens in the Russian Federation ». (in Russ.) http://publication.pravo.gov.ru/Document/View/0001202111190015 / Ссылка активна на 25.12.2023.Федеральный закон от 21.11.2011 № 323-ФЗ «Об основах охраны здоровья граждан в Российской Федерации»Federal Law of November 21, 2011 No. 323-FZ “On the fundamentals of protecting the health of citizens in the Russian Federation”. (in Russ.) http://publication.pravo.gov.ru/document/0001201111220007 / Ссылка активна на 25.12.2023.Neunert C., Terrell D.R., Arnold D.M., Buchanan G., Cines D.B., Cooper N. et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv 2019; 3(23): 3829–3866. DOI: 10.1182/bloodadvances.2019000966Neunert C., Terrell D.R., Arnold D.M., Buchanan G., Cines D.B., Cooper N. et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv 2019; 3(23): 3829–3866. DOI: 10.1182/bloodadvances.2019000966Bennett C.M., Neunert C., Grace R.F., Buchanan G., Imbach P., Vesely S.K., Kuhne T. Predictors of remission in children with newly diagnosed immune thrombocytopenia: Data from the Intercontinental Cooperative ITP Study Group Registry II participants. Pediatr Blood Cancer 2018; 65(1). DOI: 10.1002/pbc.26736Bennett C.M., Neunert C., Grace R.F., Buchanan G., Imbach P., Vesely S.K., Kuhne T. Predictors of remission in children with newly diagnosed immune thrombocytopenia: Data from the Intercontinental Cooperative ITP Study Group Registry II participants. Pediatr Blood Cancer 2018; 65(1). DOI: 10.1002/pbc.26736Hsieh Y.L., Lin L.H. Thrombocytopenic purpura following vaccination in early childhood: experience of a medical center in the past 2 decades. J Chin Med Assoc 2010; 73(12): 634–637. DOI: 10.1016/S1726–4901(10)70138–6Hsieh Y.L., Lin L.H. Thrombocytopenic purpura following vaccination in early childhood: experience of a medical center in the past 2 decades. J Chin Med Assoc 2010; 73(12): 634–637. DOI: 10.1016/S1726–4901(10)70138–6Faki Osman M.E. Childhood immune thrombocytopenia: Clinical presentation and management. Sudan J Paediatr 2012; 12(1): 27–39Faki Osman M.E. Childhood immune thrombocytopenia: Clinical presentation and management. Sudan J Paediatr 2012; 12(1): 27–39Arnold D.M. Bleeding complications in immune thrombocytopenia. Hematology Am Soc Hematol Educ Program 2015; 2015: 237–242. DOI: 10.1182/asheducation-2015.1.237Arnold D.M. Bleeding complications in immune thrombocytopenia. Hematology Am Soc Hematol Educ Program 2015; 2015: 237–242. DOI: 10.1182/asheducation-2015.1.237Provan D., Semple J.W. Recent advances in the mechanisms and treatment of immune thrombocytopenia. EBioMedicine 2022; 76: 103820. DOI: 10.1016/j.ebiom.2022.103820Provan D., Semple J.W. Recent advances in the mechanisms and treatment of immune thrombocytopenia. EBioMedicine 2022; 76: 103820. DOI: 10.1016/j.ebiom.2022.103820Witmer C.M., Lambert M.P., O’Brien S.H., Neunert C. Multicenter Cohort Study Comparing U.S. Management of Inpatient Pediatric Immune Thrombocytopenia to Current Treatment Guidelines. Pediatr Blood Cancer 2016; 63(7): 1227–1231. DOI: 10.1002/pbc.25961Witmer C.M., Lambert M.P., O’Brien S.H., Neunert C. Multicenter Cohort Study Comparing U.S. Management of Inpatient Pediatric Immune Thrombocytopenia to Current Treatment Guidelines. Pediatr Blood Cancer 2016; 63(7): 1227–1231. DOI: 10.1002/pbc.25961Consolini R., Costagliola G., Spatafora D. The Centenary of Immune Thrombocytopenia-Part 2: Revising Diagnostic and Therapeutic Approach. Front Pediatr 2017; 5: 179. DOI: 10.3389/fped.2017.00179Consolini R., Costagliola G., Spatafora D. The Centenary of Immune Thrombocytopenia-Part 2: Revising Diagnostic and Therapeutic Approach. Front Pediatr 2017; 5: 179. DOI: 10.3389/fped.2017.00179Provan D., Stasi R., Newland A.C., Blanchette V.S., Bolton-Maggs P., Bussel J.B. et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 2010; 115(2): 168–186. DOI: 10.1182/blood-2009–06–225565Provan D., Stasi R., Newland A.C., Blanchette V.S., Bolton-Maggs P., Bussel J.B. et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 2010; 115(2): 168–186. DOI: 10.1182/blood-2009–06–225565Carcao M.D., Zipursky A., Butchart S., Leaker M., Blanchette V.S. Short-course oral prednisone therapy in children presenting with acute immune thrombocytopenic purpura (ITP). Acta Paediatr 1998; 424: 71–74. DOI: 10.1111/j.1651–2227.1998.tb01239.xCarcao M.D., Zipursky A., Butchart S., Leaker M., Blanchette V.S. Short-course oral prednisone therapy in children presenting with acute immune thrombocytopenic purpura (ITP). Acta Paediatr 1998; 424: 71–74. DOI: 10.1111/j.1651–2227.1998.tb01239.xMatzdorff A., Meyer O., Ostermann H., Kiefel V., Eberl W., Kühne T. et al. Immune Thrombocytopenia — Current Diagnostics and Therapy: Recommendations of a Joint Working Group of DGHO, ÖGHO, SGH, GPOH, and DGTI. Oncol Res Treat 2018; 41(5): 1–30. DOI: 10.1159/000492187Matzdorff A., Meyer O., Ostermann H., Kiefel V., Eberl W., Kühne T. et al. Immune Thrombocytopenia — Current Diagnostics and Therapy: Recommendations of a Joint Working Group of DGHO, ÖGHO, SGH, GPOH, and DGTI. Oncol Res Treat 2018; 41(5): 1–30. DOI: 10.1159/000492187Terrell D.R., Neunert C.E., Cooper N., Heitink-Pollé K.M., Kruse C. et al. Immune Thrombocytopenia (ITP): Current Limitations in Patient Management. Medicina (Kaunas) 2020; 56(12): 667. DOI: 10.3390/medicina56120667Terrell D.R., Neunert C.E., Cooper N., Heitink-Pollé K.M., Kruse C. et al. Immune Thrombocytopenia (ITP): Current Limitations in Patient Management. Medicina (Kaunas) 2020; 56(12): 667. DOI: 10.3390/medicina56120667Kuhne T., Freedman J., Semple J.W., Doyle J., Butchart S., Blanchette V.S. Platelet and immune responses to oral cyclic dexamethasone therapy in childhood chronic immune thrombocytopenic purpura. J Pediatr 1997; 130(1): 17–24. DOI: 10.1016/S0022–3476(97)70305–6Kuhne T., Freedman J., Semple J.W., Doyle J., Butchart S., Blanchette V.S. Platelet and immune responses to oral cyclic dexamethasone therapy in childhood chronic immune thrombocytopenic purpura. J Pediatr 1997; 130(1): 17–24. DOI: 10.1016/S0022–3476(97)70305–6Beck C.E., Nathan P.C., Parkin P.C., Blanchette V.S., Macarthur C. Corticosteroids versus intravenous immune globulin for the treatment of acute immune thrombocytopenic purpura in children: a systematic review and meta-analysis of randomized controlled trials. J Pediatr 2005; 147(4): 521–527. DOI: 10.1016/j.jpeds.2005.04.032Beck C.E., Nathan P.C., Parkin P.C., Blanchette V.S., Macarthur C. Corticosteroids versus intravenous immune globulin for the treatment of acute immune thrombocytopenic purpura in children: a systematic review and meta-analysis of randomized controlled trials. J Pediatr 2005; 147(4): 521–527. DOI: 10.1016/j.jpeds.2005.04.032

The authors declare that there are no conflicts of interest present.