uzspbgmuУчёные записки Первого Санкт-Петербургского государственного медицинского университета имени академика И. П. ПавловаThe Scientific Notes of the Pavlov University1607-41812541-8807Academician I.P. Pavlov First St. Petersburg State Medical University10.24884/1607-4181-2016-23-1-43-46uzspbgmu-178Research ArticleОРИГИНАЛЬНЫЕ РАБОТЫORIGINAL PAPERSПрименение ингибитора JAK1 и JAK2 руксолитиниба в качестве пред- и посттрансплантационной терапии па% циентов с миелофиброзомApplication of inhibitor JAK1 and JAK2 ruxolitinib as pre- and posttransplant therapy in patients with myelofibrosisБарабанщиковаМ. В.BarabanshchikovaM. V.МорозоваЕ. В.MorozovaE. V.ВласоваЮ. Ю.VlasovaYu. Yu.БайковВ. В.BaykovV. V.ЗубаровскаяЛ. С.ZubarovskayaL. S.АфанасьевБ. В.AfanasyevB. V.Первый СанктПетербургский государственный медицинский университет имени академика И. П. ПавловаРоссияPavlov First State Medical University of St. PetersburgRussian Federation2016300320162314346Copyright © Барабанщикова М.В., Морозова Е.В., Власова Ю.Ю., Байков В.В., Зубаровская Л.С., Афанасьев Б.В., 20162016Барабанщикова М.В., Морозова Е.В., Власова Ю.Ю., Байков В.В., Зубаровская Л.С., Афанасьев Б.В.Barabanshchikova M.V., Morozova E.V., Vlasova Y.Y., Baykov V.V., Zubarovskaya L.S., Afanasyev B.V.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.sci-notes.ru/jour/article/view/178

Первичный миелофиброз (ПМФ) – хроническое Ph-негативное миелопролиферативное заболевание, характеризующееся, как правило, непрерывно прогрессирующим течением. АллоТГСК в настоящий момент является единственным методом терапии, позволяющим излечить больных ПМФ. Однако большая часть пациентов на момент алло-ТГСК находится в активной фазе заболевания. Использование ингибиторов JAK позволяет уменьшить объем опухолевой массы перед аллоТГСК. Проанализирован опыт применения руксолитиниба в качестве пред и посттрансплантационной терапии пациентов с ПМФ, а также больных с истинной полицитемией и эссенциальной тромбоцитемией с трансформацией в миелофиброз.

Primary myelofibrosis (PMF) is а chronic BCRABL–negative myeloproliferative disorder with progressive clinical course. Allogeneic stem cell transplantation is the only treatment optionin patients with PMF with curative potential. Most of PMF patients have active disease phase at the moment of alloHSCT. The Januskinase inhibitor ruxolitinib is effective in decreasingof tumor mass before alloHSCT. Here we report our experience in administration of ruxolitinib as pre and posttransplant therapy of patients with PMF as well as patients with polycythemia vera and essential thrombocythaemia with transformation into myelofibrosis.

миелофиброзингибитор Янус-киназруксолитинибалло-ТГСК.myelofibrosisJanuskinase inhibitorruxolitiniballo-HSCTReferencesAkpek G., Pasquini M. C., Logan B. et al. Effects of spleen status on early outcomes after hematopoietic cell transplantation// Bone Marrow Transplant. –2013. –№ 48(6). –Р. 825–831.Akpek G., Pasquini M. C., Logan B. et al. Effects of spleen status on early outcomes after hematopoietic cell transplantation// Bone Marrow Transplant. –2013. –№ 48(6). –Р. 825–831.Ballen K. K., Shrestha S., Sobocinski K. A. et al. Outcome of Transplantation for Myelofibrosis// Biol. Blood Marrow Transplant. –2010. –№ 16(3). –Р. 358–367.Ballen K. K., Shrestha S., Sobocinski K. A. et al. Outcome of Transplantation for Myelofibrosis// Biol. Blood Marrow Transplant. –2010. –№ 16(3). –Р. 358–367.Bradley E. C., EricksonViitanen S., Vaddi K.et al. Safety and Efficacy of INCB018424, a JAK1 and JAK2 Inhibitor, in Myelofibrosis// NEJM. –2010. –№ 362(15). –Р. 1117–1127.Bradley E. C., EricksonViitanen S., Vaddi K.et al. Safety and Efficacy of INCB018424, a JAK1 and JAK2 Inhibitor, in Myelofibrosis// NEJM. –2010. –№ 362(15). –Р. 1117–1127.Choi J.L., Alahmari B. et al. Pharmacologic blockade of JAK1/JAK2 reduces GvHD and preserves the graftversusleukemia effect// PLoS One. –2014. – № 9(10). –Р. 1117–1127.Choi J.L., Alahmari B. et al. Pharmacologic blockade of JAK1/JAK2 reduces GvHD and preserves the graftversusleukemia effect// PLoS One. –2014. – № 9(10). –Р. 1117–1127.Eswar T., Saranya K. Experience with Administration of Ruxolitinib after Allogeneic Stem Cell Transplantation (alloSCT) in Patients with Myelofibrosis// Biol. Blood Marrow. Transpl. – 2015. – № 21. –Р. 315.Eswar T., Saranya K. Experience with Administration of Ruxolitinib after Allogeneic Stem Cell Transplantation (alloSCT) in Patients with Myelofibrosis// Biol. Blood Marrow. Transpl. – 2015. – № 21. –Р. 315.Gangat N., Caramazza D., Vaidya R. et al. DIPSS Plus?: A Refined Dynamic International Prognostic Scoring System for Primary Myelofibrosis That Incorporates Prognostic Information From Karyotype, Platelet Count and Transfusion Status // JCO. – 2011. – № 29(4). –Р. 392–397.Gangat N., Caramazza D., Vaidya R. et al. DIPSS Plus?: A Refined Dynamic International Prognostic Scoring System for Primary Myelofibrosis That Incorporates Prognostic Information From Karyotype, Platelet Count and Transfusion Status // JCO. – 2011. – № 29(4). –Р. 392–397.Heine A., Andrea S., Held E. et al. The JAKinhibitor ruxolitinib impairs dendritic cell function in vitro and in vivo // Blood. – 2013. – № 122(7). –Р. 1192–1203.Heine A., Andrea S., Held E. et al. The JAKinhibitor ruxolitinib impairs dendritic cell function in vitro and in vivo // Blood. – 2013. – № 122(7). –Р. 1192–1203.Kroger N., Holler E., Kobbe G. et al. Allogeneic stem cell transplantation after reducedintensity conditioning in patients with myelofibrosis: a prospective, multicenter study of the Chronic Leukemia Working Party of the European Group for Blood and Marrow Transplantation// Blood.– 2009. – № 114(26). – Р. 5264–5270.Kroger N., Holler E., Kobbe G. et al. Allogeneic stem cell transplantation after reducedintensity conditioning in patients with myelofibrosis: a prospective, multicenter study of the Chronic Leukemia Working Party of the European Group for Blood and Marrow Transplantation// Blood.– 2009. – № 114(26). – Р. 5264–5270.Kvasnicka H. M., Van Der Walt J. European consensus on grading bone marrow fibrosis and assessment of cellularity//Haematologica. –2005. – № 90. –Р. 1128–1132.Kvasnicka H. M., Van Der Walt J. European consensus on grading bone marrow fibrosis and assessment of cellularity//Haematologica. –2005. – № 90. –Р. 1128–1132.Przepiorka K. H., Weisdorf D., Martin P. Consensus Conference on Acute GVHD Grading// Bone Marrow. Transplant. –1995. – № 15(6). –Р. 825–828.Przepiorka K. H., Weisdorf D., Martin P. Consensus Conference on Acute GVHD Grading// Bone Marrow. Transplant. –1995. – № 15(6). –Р. 825–828.Robin M. Ruxolitinib Before Allogeneic Hematopoietic Stem Cell Transplantation (HSCT) In Patients With myelofibrosis?: a Preliminary Descriptive Report Of The JAK ALLO Study, a Phase II Trial Sponsored By GoelamsFIM In Collaboration With The Sfgmtc //ASH. –2013. – Abstract 306.Robin M. Ruxolitinib Before Allogeneic Hematopoietic Stem Cell Transplantation (HSCT) In Patients With myelofibrosis?: a Preliminary Descriptive Report Of The JAK ALLO Study, a Phase II Trial Sponsored By GoelamsFIM In Collaboration With The Sfgmtc //ASH. –2013. – Abstract 306.Rondelli D., Goldberg J. D., Isola L. et al. MPDRC 101 prospective study of reducedintensity allogeneic hematopoietic stem cell transplantation in patients with myelofibrosis // Blood. – 2014. – № 124(7). –Р. 1183–1191.Rondelli D., Goldberg J. D., Isola L. et al. MPDRC 101 prospective study of reducedintensity allogeneic hematopoietic stem cell transplantation in patients with myelofibrosis // Blood. – 2014. – № 124(7). –Р. 1183–1191.Shanavas M., Popat U. Outcomes of Allogeneic Hematopoietic Cell Transplantation in Patients with Myelofibrosis with Prior Exposure to Janus Kinase 1/2 Inhibitors// Biol. Blood Marrow. Transpl. –2016. – № 22. –Р. 432–440.Shanavas M., Popat U. Outcomes of Allogeneic Hematopoietic Cell Transplantation in Patients with Myelofibrosis with Prior Exposure to Janus Kinase 1/2 Inhibitors// Biol. Blood Marrow. Transpl. –2016. – № 22. –Р. 432–440.Stubig T., Alchalby H., Ditschkowski M. et al. JAK inhibition with ruxolitinib as pretreatment for allogeneic stem cell transplantation in primary or postET/PV myelofibrosis // Leukemia. – 2014. – № 28(8). –Р. 1736–1738.Stubig T., Alchalby H., Ditschkowski M. et al. JAK inhibition with ruxolitinib as pretreatment for allogeneic stem cell transplantation in primary or postET/PV myelofibrosis // Leukemia. – 2014. – № 28(8). –Р. 1736–1738.Teo S., Tiedt R., Passweg J. R.et al. A GainofFunction Mutation of JAK2 // NEJM. –2005. –№ 352(17). –Р. 1779–1790.Teo S., Tiedt R., Passweg J. R.et al. A GainofFunction Mutation of JAK2 // NEJM. –2005. –№ 352(17). –Р. 1779–1790.Vannucchi A. M., Kantarjian H. M., Kiladjian J.J. et al. A pooled analysis of overall survival in COMFORTI and COMFORTII, 2 randomized phase III trials of ruxolitinib for the treatment of myelofibrosis // Haematologica. –2015. – № 100(9). –Р. 1139–1145.Vannucchi A. M., Kantarjian H. M., Kiladjian J.J. et al. A pooled analysis of overall survival in COMFORTI and COMFORTII, 2 randomized phase III trials of ruxolitinib for the treatment of myelofibrosis // Haematologica. –2015. – № 100(9). –Р. 1139–1145.Verstovsek S., Mesa R. A., Gotlib J. et al. Efficacy, safety, and survival with ruxolitinib in patients with myelofibrosis: results of a median 3year followup of COMFORTI// Haematologica. – 2015. – № 100(4). –Р. 479–488.Verstovsek S., Mesa R. A., Gotlib J. et al. Efficacy, safety, and survival with ruxolitinib in patients with myelofibrosis: results of a median 3year followup of COMFORTI// Haematologica. – 2015. – № 100(4). –Р. 479–488.Zeiser R., Burchert A., Lengerke C. et al. Ruxolitinib in corticosteroidrefractory graftversushost disease after allogeneic stem cell transplantation: a multicenter survey // Leukemia. – 2015. –№ 29(10). –Р. 2062–2068.Zeiser R., Burchert A., Lengerke C. et al. Ruxolitinib in corticosteroidrefractory graftversushost disease after allogeneic stem cell transplantation: a multicenter survey // Leukemia. – 2015. –№ 29(10). –Р. 2062–2068.

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