OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press3510.17650/1818-8346-2013-8-2-61-69PHARMACOTHERAPYФАРМАКОТЕРАПИЯImmune thrombocytopenia: clinical manifestation and therapy response. The interim analysis of Russian register of patients with primary immune thrombocytopenia and literature reviewИммунная тромбоцитопения: клинические проявления и ответ на терапию. Промежуточный анализ данных Российского регистра пациентов с первичной иммунной тромбоцитопенией и обзор литературыLisukovI. A.ЛисуковИ. А.
Russian Federation
igor_lisukov@mail.ruMaschanA. A.МасчанА. А.
Russian Federation
ShamardinaA. V.ШамардинаА. В.
Russian Federation
ChagorovaT. V.ЧагороваТ. В.
Russian Federation
DavydkinI. L.ДавыдкинИ. Л.
Russian Federation
SychevaT. M.СычеваТ. М.
Russian Federation
ZagoskinaT. P.ЗагоскинаТ. П.
Russian Federation
KaryaginaE. V.КарягинаЕ. В.
Russian Federation
SalogubG. N.СалогубГ. Н.
Russian Federation

кафедра факультетской терапии

SavinovaM. T.СавиноваМ. Т.
Russian Federation
ShelekhovaT. V.ШелеховаТ. В.
Russian Federation

клиника гематологии

KovalevaL. G.КовалеваЛ. Г.
Russian Federation
ShneyderT. V.ШнейдерТ. В.
Russian Federation
UnzhekovaA. N.УнжековаА. Н.
Russian Federation
KuznetsovaE. E.КузнецоваЕ. Е.
Russian Federation
ShatokhinYu. V.ШатохинЮ. В.
Russian Federation
IvanovaM. O.ИвановаМ. О.
Russian Federation

Clinical Diagnostic Center “Clinics”

консультационно-диагностический центр

VinogradovaE. Yu.ВиноградоваЕ. Ю.
Russian Federation
VolodichevaE. M.ВолодичеваЕ. М.
Russian Federation
MarkovaI. V.МарковаИ. В.
Russian Federation
KanyukovaO. V.КанюковаО. В.
Russian Federation
AbdulkadyrovK. M.АбдулкадыровК. М.
Russian Federation
SedlovaYu. A.СедловаЮ. А.
Russian Federation
OsyunikhinaS. M.ОсюнихинаС. М.
Russian Federation
KaplanovK. D.КаплановК. Д.
Russian Federation
TsvetaevaN. V.ЦветаеваН. В.
Russian Federation
AkhmadeevA. R.АхмадеевА. Р.
Russian Federation
UspenskayaO. S.УспенскаяО. С.
Russian Federation
KulaginA. D.КулагинА. Д.
Russian Federation
MedvedevaN. V.МедведеваН. В.
Russian Federation
RumyantsevA. G.РумянцевА. Г.
Russian Federation
AfanasievB. V.АфанасьевБ. В.
Russian Federation
Raisa Gorbacheva Memorial Institute of Children Hematology and Transplantation, St.-Petersburg I.P. Pavlov State Medical University, Ministry of Health of RussiaИнститут детской гематологии и трансплантологии им. Р.М. Горбачевой ГБОУ ВПО СПбГМУ им. акад. И.П. Павлова» Минздрава РоссииDmitriy Rogachev Federal Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaФГБУ ФНКЦ ДГОИ им. Дмитрия Рогачева Минздрава РоссииRegional Children Clinical HospitalГУ «Нижегородская детская областная клиническая больница», Нижний НовгородRegional Oncologic DispensaryГБУЗ «Областной онкологический диспансер»Samara State Medical University, Ministry of Health of RussiaГБОУ ВПО «Самарский государственный медицинский университет» Минздрава РоссииAlexander-and-Mary Regional Clinical HospitalГБУЗ АО «Александро-Мариинская областная клиническая больница»Kirov Research Centre of Hematology and Transfusiology, Federal Medical Biological AgencyФГБУН «Кировский научно-исследовательский институт гематологии и переливания крови» ФМБА РоссииCity Clinical Hospital № 15МУЗ «Городская клиническая больница № 15»St.-Petersburg I.P. Pavlov State Medical University, Ministry of Health of RussiaГБОУ ВПО СПбГМУ им. акад. И.П. Павлова Минздрава РоссииCity Clinical Hospital № 16ГАУЗ «Городская клиническая больница № 16»Hematology Clinic, V.I. Razumovskiy Saratov State Medical UniversityГБОУ ВПО «Саратовский государственный медицинский университет им. В.И. Разумовского» Минздрава РоссииHematological Research Centre, Ministry of Health of RussiaФГБУ «Гематологический научный центр» Минздрава РоссииLeningrad Regional Clinical HospitalГУЗ «Ленинградская областная клиническая больница»Perm Territorial Clinical HospitalГБУЗ ПК «Пермская краевая клиническая больница»Regional Clinical HospitalГБУЗ «Оренбургская областная клиническая больница»Rostov State Medical University, Ministry of Health of RussiaГБОУ ВПО «Ростовский государственный медицинский университет» Минздрава РоссииI.P. Pavlov State Medical University, Ministry of Health of RussiaКлиника ГБОУ ВПО СПбГМУ им. акад. И.П. Павлова Минздрава РоссииTerritorial Clinical HospitalКГБУЗ «Краевая клиническая больница»Regional Clinical HospitalГУЗ ТО «Тульская областная клиническая больница»Clinical Medical Unit № 1МУЗ «Клиническая медико-санитарная часть № 1»Russian Research Institute of Hematology and Transfusiology, Russian Federal Medico-biological AgencyФГБУ РосНИИГТ ФМБА РоссииRegional Clinical HospitalБУ ХМАО-Югры «Сургутская окружная клиническая больница»City Clinical Hospital № 4МУЗ «Городская клиническая больница № 4»Regional Oncologic Clinical Dispensary № 1ГБУЗ «Волгоградский областной онкологический клинический диспансер № 1»Republic Clinical HospitalГУЗ «Республиканская клиническая больница» Министерства здравоохранения Республики ТатарстанRaisa Gorbacheva Memorial Institute of Children Hematology and Transplantation, St.-Petersburg I.P. Pavlov State Medical University, Ministry of Health of RussiaИнститут детской гематологии и трансплантологии им. Р.М. Горбачевой ГБОУ ВПО СПбГМУ им. акад. И.П. Павлова» Минздрава РоссииCity Hospital № 31МУЗ «Городская клиническая больница № 31»2205201382616921072014210720142013https://oncohematology.abvpress.ru/ongm/article/view/35

Primary immune thrombocytopenia (ITP) is a rare (orphan) blood disease. Most frequent manifestations of ITP are purpura, petechiae and bleedings with many patients have either no symptoms or minimal bleedings manifestation. The management of ITP varies widely and must be based on current international recommendations and individual assessment of clinical course. The paper presents the results of interim analysis of clinical course and therapeutic approaches in the Russian register of ITP patients with immune thrombocytopenia and literature review about ITP treatment approaches.

Первичная иммунная тромбоцитопения (ИТП) относится к редким (орфанным) заболеваниям. Наиболее частыми проявлениями ИТП являются пурпура, петехии и кровотечения, при этом многие пациенты не имеют клинических симптомов заболевания или отмечают минимальные проявления кровоточивости. В последние годы были опубликованы современные международные рекомендации по лечению ИТП, при этом подход к лечению каждого конкретного пациента всегда определяется на основе индивидуальной оценки течения заболевания. В данной статье представлены результаты промежуточного анализа особенностей клинического течения и терапевтических подходов к ИТП по данным Российского регистра пациентов с иммунной тромбоцитопенией, а также обзор данных литературы по лечению ИТП.

immune thrombocytopeniaregistryclinical manifestationtreatmentиммунная тромбоцитопениярегистрклинические проявлениялечение1. Abrahamson P.E., Hall S.A., Feudjo-Tepie M. et al. The incidence of idiopathic thrombocytopenic purpura (ITP) among adults: a population-based study and literature review. Eur J Haematol 2009;83:83–9.Abrahamson P.E., Hall S.A., Feudjo-Tepie M. et al. The incidence of idiopathic thrombocytopenic purpura (ITP) among adults: a population-based study and literature review. Eur J Haematol 2009;83:83–9.2. Aoki T., Harada Y., Matsubara E. et al. Thrombopoietin receptor agonists in refractory immune thrombocytopenia: differential responses to eltrombopag and romiplostim: a case report and possible explanations. J Clin Pharm Ther 2012 Dec;37(6):729–32.Aoki T., Harada Y., Matsubara E. et al. Thrombopoietin receptor agonists in refractory immune thrombocytopenia: differential responses to eltrombopag and romiplostim: a case report and possible explanations. J Clin Pharm Ther 2012 Dec;37(6):729–32.3. Arnold D.M., Dentali F., Crowther M.A. et al. Systematic review: efficacy and safety of rituximab for adults with idiopathic thrombocytopenic purpura. Ann Intern Med 2007;146(1):25–33.Arnold D.M., Dentali F., Crowther M.A. et al. Systematic review: efficacy and safety of rituximab for adults with idiopathic thrombocytopenic purpura. Ann Intern Med 2007;146(1):25–33.4. Arnold D.M., Heddle N.M., Carruthers J. et al. A pilot randomized trial of adjuvant rituximab or placebo for non-splenectomized patients with immune thrombocytopenia. Blood 2012 Feb 9;119(6):1356–62.Arnold D.M., Heddle N.M., Carruthers J. et al. A pilot randomized trial of adjuvant rituximab or placebo for non-splenectomized patients with immune thrombocytopenia. Blood 2012 Feb 9;119(6):1356–62.5. Bizzoni L., Mazzucconi M., Gentile M. et al. Idiopathic thrombocytopenic purpura (ITP) in the eldery: clinical course in 178 patients. Eur J Haematol 2006;76(3):210–6. 6. Boccia R., Kuter D.J., Rummel M.J. et al. The effects of romiplostim or standard of care (SOC) on splenectomy and treatment failure of patients who had immune thrombocytopenia (ITP) for less than or equal to one year. Blood 2010;116(21):3702.Bizzoni L., Mazzucconi M., Gentile M. et al. Idiopathic thrombocytopenic purpura (ITP) in the eldery: clinical course in 178 patients. Eur J Haematol 2006;76(3):210–6. 6. Boccia R., Kuter D.J., Rummel M.J. et al. The effects of romiplostim or standard of care (SOC) on splenectomy and treatment failure of patients who had immune thrombocytopenia (ITP) for less than or equal to one year. Blood 2010;116(21):3702.7. Bussel J.B., Kuter D.J., Pullarkat V. et al. Safety and efficacy of long-term treatment with romiplostim in thrombocytopenic patients with chronic ITP. Blood 2009;113:2161–71.Bussel J.B., Kuter D.J., Pullarkat V. et al. Safety and efficacy of long-term treatment with romiplostim in thrombocytopenic patients with chronic ITP. Blood 2009;113:2161–71.8. Bussel J., Rodeghiero F., Lyons R.M. et al. Sustained hemostatic platelet counts in adults with immune thrombocytopenia (ITP) following cessation of treatment with the TPO receptor agonist romiplostim: report of 9 cases. Blood 2011;118(21):3281.Bussel J., Rodeghiero F., Lyons R.M. et al. Sustained hemostatic platelet counts in adults with immune thrombocytopenia (ITP) following cessation of treatment with the TPO receptor agonist romiplostim: report of 9 cases. Blood 2011;118(21):3281.9. Chen J., Herceg-Harjacek L., Groopman J., Grabarek J. Regulation of platelet activation in vitro by the c-Mpl ligand, thrombopoietin. Blood1995;86:4054–62.Chen J., Herceg-Harjacek L., Groopman J., Grabarek J. Regulation of platelet activation in vitro by the c-Mpl ligand, thrombopoietin. Blood1995;86:4054–62.10. Cheng G., Saleh M.N., Marcher C. et al. Eltrombopag for management of chronic immune thrombocytopenia (RAISE): a 6-month, randomised, phase 3 study. Lancet 2011;377:393–402.Cheng G., Saleh M.N., Marcher C. et al. Eltrombopag for management of chronic immune thrombocytopenia (RAISE): a 6-month, randomised, phase 3 study. Lancet 2011;377:393–402.11. Cooper K.L., Fitzgerald P., Dillingham K. et al. Romiplostim and eltrombopag for immune thrombocytopenia: methods for indirect comparison. Int J Technol Assess Health Care 2012;28(3):Cooper K.L., Fitzgerald P., Dillingham K. et al. Romiplostim and eltrombopag for immune thrombocytopenia: methods for indirect comparison. Int J Technol Assess Health Care 2012;28(3):249–58.–58.12. Cooper N., Stasi R., Cunningham- Rundles S. et al. The efficacy and safety of B-cell depletion with anti-CD20 monoclonal antibody in adults with chronic immune thrombocytopenic purpura. Br J Haematol 2004;125(2):232–9.Cooper N., Stasi R., Cunningham- Rundles S. et al. The efficacy and safety of B-cell depletion with anti-CD20 monoclonal antibody in adults with chronic immune thrombocytopenic purpura. Br J Haematol 2004;125(2):232–9.13. Fogarty P.F. Chronic immune thrombocytopenia in adults: epidemiology and clinical presentation. Hematol Oncol Clin N Am 2009;23:1213–21.Fogarty P.F. Chronic immune thrombocytopenia in adults: epidemiology and clinical presentation. Hematol Oncol Clin N Am 2009;23:1213–21.14. Frederiksen H., Schmidt K. The incidence of idiopathic thrombocytopenic purpura in adults increases with age. Blood 1999;94(3):909–13.Frederiksen H., Schmidt K. The incidence of idiopathic thrombocytopenic purpura in adults increases with age. Blood 1999;94(3):909–13.15. Godeau B., Porcher R., Fain O. et al. Rituximab efficacy and safety in adults splenectomy candidates with chronic immune thrombocytopenic purpura: results of a prospective multicenter phase 2 study. Blood 2008;112(4):999–1004.Godeau B., Porcher R., Fain O. et al. Rituximab efficacy and safety in adults splenectomy candidates with chronic immune thrombocytopenic purpura: results of a prospective multicenter phase 2 study. Blood 2008;112(4):999–1004.16. Jenssens A., Tarantino M., Bird R. et al. Final results from an international, multi-center, single-arm study evaluating the safety and efficacy of romiplostim in adults with primary immune thrombocytopenia (ITP). Blood 2011;118(21):3279.Jenssens A., Tarantino M., Bird R. et al. Final results from an international, multi-center, single-arm study evaluating the safety and efficacy of romiplostim in adults with primary immune thrombocytopenia (ITP). Blood 2011;118(21):3279.17. Kojouri K., Vesely S.K., Terrell D.R. et al. Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response and surgical complications Blood 2004;104(9):2623–34.Kojouri K., Vesely S.K., Terrell D.R. et al. Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response and surgical complications Blood 2004;104(9):2623–34.18. Kuter D.J., Bussel J.B., Lyons R.M. et al. Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: a double-blind randomized controlledtrial. Lancet 2008;371(9610):395–403.Kuter D.J., Bussel J.B., Lyons R.M. et al. Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: a double-blind randomized controlledtrial. Lancet 2008;371(9610):395–403.19. Kuter D., Rummel M., Boccia R. et al. Romiplostim or standart of care in patients with immune thrombocytopenia. N Engl J Med 2010;363(20):1889–99.Kuter D., Rummel M., Boccia R. et al. Romiplostim or standart of care in patients with immune thrombocytopenia. N Engl J Med 2010;363(20):1889–99.20. McMillan R., Durette C. Long-term outcomes in adults with chronic ITP after splenectomy failure. Blood 2004;104(4):956–60.McMillan R., Durette C. Long-term outcomes in adults with chronic ITP after splenectomy failure. Blood 2004;104(4):956–60.21. Neylon A.J., Saunders P.W., Haward M.R. et al. Clinically significant newly presenting autoimmuneNeylon A.J., Saunders P.W., Haward M.R. et al. Clinically significant newly presenting autoimmunethrombocytopenic purpura in adults: a prospective study of population-based cohort of 245 patients. Br J Haematol 2003;122(6):966–74.22. Patel V.L., Mahevas M., Lee S. Y. et al. Outcomes 5 years after response to rituximab therapy in children and adults with mmune thrombocytopenia.Blood 2012;119:5989–95.Patel V.L., Mahevas M., Lee S. Y. et al. Outcomes 5 years after response to rituximab therapy in children and adults with mmune thrombocytopenia.Blood 2012;119:5989–95.23. Portielje J.E., Westendorp R.G., Kluin- Nelemans H.C., Brand A. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood 2001;97(9):2549–54.Portielje J.E., Westendorp R.G., Kluin- Nelemans H.C., Brand A. Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood 2001;97(9):2549–54.24. Rodeghiero F., Stasi R., Gernsheimer T. et al. Standartization of terminology definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international woring group. Blood 2009;113(11):2386–93.Rodeghiero F., Stasi R., Gernsheimer T. et al. Standartization of terminology definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international woring group. Blood 2009;113(11):2386–93.25. Vianelly N., Galli M., de Vivo A. et al. Gruppo Italiano per lo Studio delle Malattie Ematologiche dell`Adulto. Efficacy and safety of splenectomy in immune thrombocytopenic purpura: long-term results of 402 cases. Haematologica 2005; 90(1):72–7.Vianelly N., Galli M., de Vivo A. et al. Gruppo Italiano per lo Studio delle Malattie Ematologiche dell`Adulto. Efficacy and safety of splenectomy in immune thrombocytopenic purpura: long-term results of 402 cases. Haematologica 2005; 90(1):72–7.26. Wasser J., Boccia R., Lyons R.M. Use of rituximab in a study comparing the thrombopoietin mimetic romiplostim with standard of care (SOC) in a patients with immune thrombocytopenia (ITP). Blood 2011;118(21):3282.Wasser J., Boccia R., Lyons R.M. Use of rituximab in a study comparing the thrombopoietin mimetic romiplostim with standard of care (SOC) in a patients with immune thrombocytopenia (ITP). Blood 2011;118(21):3282.27. Wong G.C., Lee L.H. A study of idiopathic thrombocytopenic purpura (ITP) patients over a ten-year period. Ann Acad Med Singapore 1998;27(6):789–93.Wong G.C., Lee L.H. A study of idiopathic thrombocytopenic purpura (ITP) patients over a ten-year period. Ann Acad Med Singapore 1998;27(6):789–93.