Management of patients with immune thrombocytopenia in the Moscow region

Background. Idiopathic thrombocytopenic purpura (ITp) is an autoimmune disease characterized by antibody-mediated platelets destruction and impairment of their production, which manifests itself as: isolated thrombocytopenia, risk of spontaneous hemorrhage and bleeding of varying severity. ITp is a...

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Main Authors: S. G. Zakharov, T. A. Mitina, R. V. Vardanyan, I. N. Kontievskiy, A. P. Faenko, Z. R. Tekeeva
Format: Article
Language:Russian
Published: ABV-press 2022-11-01
Series:Онкогематология
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Online Access:https://oncohematology.abvpress.ru/ongm/article/view/586
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author S. G. Zakharov
T. A. Mitina
R. V. Vardanyan
I. N. Kontievskiy
A. P. Faenko
Z. R. Tekeeva
author_facet S. G. Zakharov
T. A. Mitina
R. V. Vardanyan
I. N. Kontievskiy
A. P. Faenko
Z. R. Tekeeva
author_sort S. G. Zakharov
collection DOAJ
description Background. Idiopathic thrombocytopenic purpura (ITp) is an autoimmune disease characterized by antibody-mediated platelets destruction and impairment of their production, which manifests itself as: isolated thrombocytopenia, risk of spontaneous hemorrhage and bleeding of varying severity. ITp is a hematological, orphan disease with an incidence of 1–4 cases per 100,000 population. In modern literature, primary and secondary immune thrombocytopenias are distinguished. primary immune thrombocytopenia is a diagnosis of exclusion. To verify it, a certain diagnostic search is required.Aim. To evaluate clinical characteristics and treatment efficacy in patients with a confirmed primary immune thrombocytopenia in the Moscow region.Materials and methods. This article presents the results of an analysis of more than 2,400 outpatient records of patients diagnosed with thrombocytopenia (for the period from 2010 to 2022). Of these, about 400 confirmed clinical cases of various ITp forms were included in the ITp registry of the Moscow Region. All patients live in the Moscow region, receive treatment and are observed at the Center for Orphan diseases of the M.f. vladimirskiy Moscow Regional Research Clinical Institute.Results. There are 415 patients with a verified diagnosis of ITp in the register of the Moscow Region Center for Orphan diseases of the M.f. vladimirskiy Moscow Regional Research Clinical Institute (71 % (n = 294) are female). In 69.8 % (n = 290) of patients at the time of disease manifestation, hemorrhagic syndrome was recorded. As a first-line therapy, 92.8 % (n = 385) of patients received corticosteroids (prednisolone, methylprednisolone, dexamethasone), in the second-line therapy, 82 % (n = 340) of patients were recommended therapy with thrombopoietin receptor agonists (romiplostim, eltrombopag). The options for third-line therapy in patients with ITp are rituximab monotherapy, splenectomy, and intravenous immunoglobulin. Splenectomy was performed in 3.37 % (n = 14) of patients.Conclusion. when evaluating this register, the highest efficiency of thrombopoietin receptor agonists (romiplostim, eltrombopag) is observed – 84.1 % of the objective response.
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spelling doaj-art-bd5dc8d312224c1ca4702c3c58a7e1cb2025-08-20T03:35:33ZrusABV-pressОнкогематология1818-83462413-40232022-11-01174333710.17650/1818-8346-2022-17-4-33-37464Management of patients with immune thrombocytopenia in the Moscow regionS. G. Zakharov0T. A. Mitina1R. V. Vardanyan2I. N. Kontievskiy3A. P. Faenko4Z. R. Tekeeva5M.F. Vladimirskiy Moscow Regional Research Clinical Institute;M.F. Vladimirskiy Moscow Regional Research Clinical Institute;M.F. Vladimirskiy Moscow Regional Research Clinical Institute;M.F. Vladimirskiy Moscow Regional Research Clinical Institute;M.F. Vladimirskiy Moscow Regional Research Clinical Institute;M.F. Vladimirskiy Moscow Regional Research Clinical Institute;Background. Idiopathic thrombocytopenic purpura (ITp) is an autoimmune disease characterized by antibody-mediated platelets destruction and impairment of their production, which manifests itself as: isolated thrombocytopenia, risk of spontaneous hemorrhage and bleeding of varying severity. ITp is a hematological, orphan disease with an incidence of 1–4 cases per 100,000 population. In modern literature, primary and secondary immune thrombocytopenias are distinguished. primary immune thrombocytopenia is a diagnosis of exclusion. To verify it, a certain diagnostic search is required.Aim. To evaluate clinical characteristics and treatment efficacy in patients with a confirmed primary immune thrombocytopenia in the Moscow region.Materials and methods. This article presents the results of an analysis of more than 2,400 outpatient records of patients diagnosed with thrombocytopenia (for the period from 2010 to 2022). Of these, about 400 confirmed clinical cases of various ITp forms were included in the ITp registry of the Moscow Region. All patients live in the Moscow region, receive treatment and are observed at the Center for Orphan diseases of the M.f. vladimirskiy Moscow Regional Research Clinical Institute.Results. There are 415 patients with a verified diagnosis of ITp in the register of the Moscow Region Center for Orphan diseases of the M.f. vladimirskiy Moscow Regional Research Clinical Institute (71 % (n = 294) are female). In 69.8 % (n = 290) of patients at the time of disease manifestation, hemorrhagic syndrome was recorded. As a first-line therapy, 92.8 % (n = 385) of patients received corticosteroids (prednisolone, methylprednisolone, dexamethasone), in the second-line therapy, 82 % (n = 340) of patients were recommended therapy with thrombopoietin receptor agonists (romiplostim, eltrombopag). The options for third-line therapy in patients with ITp are rituximab monotherapy, splenectomy, and intravenous immunoglobulin. Splenectomy was performed in 3.37 % (n = 14) of patients.Conclusion. when evaluating this register, the highest efficiency of thrombopoietin receptor agonists (romiplostim, eltrombopag) is observed – 84.1 % of the objective response.https://oncohematology.abvpress.ru/ongm/article/view/586idiopathic thrombocytopenic purpuraromiplostimeltrombopaghemorrhagic syndromethrombocytopenia
spellingShingle S. G. Zakharov
T. A. Mitina
R. V. Vardanyan
I. N. Kontievskiy
A. P. Faenko
Z. R. Tekeeva
Management of patients with immune thrombocytopenia in the Moscow region
Онкогематология
idiopathic thrombocytopenic purpura
romiplostim
eltrombopag
hemorrhagic syndrome
thrombocytopenia
title Management of patients with immune thrombocytopenia in the Moscow region
title_full Management of patients with immune thrombocytopenia in the Moscow region
title_fullStr Management of patients with immune thrombocytopenia in the Moscow region
title_full_unstemmed Management of patients with immune thrombocytopenia in the Moscow region
title_short Management of patients with immune thrombocytopenia in the Moscow region
title_sort management of patients with immune thrombocytopenia in the moscow region
topic idiopathic thrombocytopenic purpura
romiplostim
eltrombopag
hemorrhagic syndrome
thrombocytopenia
url https://oncohematology.abvpress.ru/ongm/article/view/586
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