OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press71310.17650/1818-8346-2009-0-1-51-5PHARMACOTHERAPYФАРМАКОТЕРАПИЯStimulation of platelet production: the new treatment approach to chronic immune thrombocytopenic purpuraСтимуляция продукции тромбоцитов: новый подход к лечению хронической иммунной тромбоцитопенической пурпурыMaschanA. A.МасчанА. А.
Russian Federation

Moscow

Москва

RumyantsevA. G.РумянцевА. Г.
Russian Federation

Moscow

Москва

Federal Research Center of Pediatric Hematology, Oncology and ImmunologyФедеральный научно-клинический центр детской гематологии, онкологии и иммунологии Минздравсоцразвития240220091515624112022241120222009https://oncohematology.abvpress.ru/ongm/article/view/713

Chronic immune thrombocytopenic purpura (ITP) is one of the most frequent immune hematological disorders in which development the leading part is played by the antibodies directed against a narrow spectrum of platelet antigens. Though death rate at chronic ITP is insignificant, no more than 1%; disease strongly reduces patients quality of a life, and frequently requires difficult and expensive treatment. Antiplatelets antibodies mediating ITP, are directed against glycoprotein GP IIb/IIIa and, less often, GP Ib/IX. At ITP can be registered both raised bone marrow platelet production and accelerated peripheral blood platelet clearance, and strongly decreased production and normal platelet life-span. Antibodies to platelet membrane glycoproteins directly disturb platelet production in bone marrow. Standard drug interventions at chronic ITP - glucocorticoids in various doses, high doses intravenous immunoglobulin, anti-D immunoglobulin, rituximab - rare lead to stable increase platelet number while in 2/3 patients splenectomy is effective. The new therapy approach in chronic ITP is stimulation of platelet production in bone marrow. Recombinant fusion protein Romiplostim stimulates trombopoietin receptor and it is effective at long-term therapy in 87% patients with chronic ITP irrespective of previous splenectomy. TPO antibodies development with romiplostim therapy was not revealed.

Хроническая иммунная тромбоцитопеническая пурпура (ИТП) является одной из самых частых иммунных гемопатий, в развитии которой ведущую роль играют антитела, направленные против узкого спектра тромбоцитарных антигенов. Несмотря на то что смертность при хронической ИТП невелика (не более 1%), заболевание драматически снижает качество жизни пациента и зачастую требует сложного и дорогого лечения. Антитромбоцитарные антитела, опосредующие ИТП, направлены против гликопротеина GP IIb/IIIa и, реже, GP Ib/IX. При ИТП могут отмечаться как повышенная костномозговая продукция тромбоцитов и ускоренный периферический клиренс, так и резко сниженная продукция при нормальном времени жизни тромбоцитов. Показано, что антитела к гликопротеинам мембраны тромбоцитов напрямую нарушают продукцию тромбоцитов мегакариоцитами. Стандартные медикаментозные вмешательства при хронической ИТП — глюкокортикоиды в различных дозах, высокие дозы внутривенного иммуноглобулина, антирезусный иммуноглобулин, ритуксимаб — редко позволяют достигать устойчивого повышения числа тромбоцитов, в то время как спленэктомия эффективна у 2/3 пациентов. Новым подходом ктерапии хронической ИТП является стимуляция продукции тромбоцитов мегакариоцитами. Генно-инженерный конструкт ромиплостим стимулирует рецептор тромбопоэтина и эффективен при длительном применении у 87% пациентов с хронической ИТП независимо от предшествующей спленэктомии. Развития антител к тромбопоэтину при применении ромиплостима не отмечалось.

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