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Институт теоретической и экспериментальной биофизики Российской академии наук.

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199406, Санкт-Петербург, ул.Гаванская, д. 49, корп.2

ISSN 1999-6314

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«
Vol. 23, Art. 38 (pp. 657-667)    |    2022       
»

Evaluation of the efficiency of the first line glucocorticosteroid therapy patients with immune thrombocytopenia according to the data of the register of the moscow region
Zakharov S. G., Mitina T. A., Vardanyan R. V., Tekeyeva Z. R., Kontievsky I. N.

GBUZ MO "Moscow Regional Research Clinical Institute named after I.I. M.F. Vladimirsky



Brief summary

Immune thrombocytopenia is an incurable chronic orphan blood disease that manifests itself as a hemorrhagic syndrome of varying severity. The etiology of the disease is unknown. The pathogenesis of the disease is characterized by the formation of antibodies to platelets and increased destruction of platelets and their precursors, megakaryocytes. The present study included 300 patients with primary newly diagnosed immune thrombocytopenic purpura. The duration of the disease is from 0-3.0 months. The study included men and women aged 18 to 87 years, median age 57 years. Women 201 (67%), median age 59.5 years, men 99 (33%), median age 54.5 years. The platelet count at admission ranged from 1 to 40 x 109/L. Patients complained of hemorrhagic syndrome of varying severity from petechial rash to ecchymosis. Some patients complained of bleeding: gingival, nasal, metro and menorrhagia. Before prednisolone therapy: in 214 patients (71.6%), the platelet level was from 0 to 29 x 109/l h. In 40 patients (13.4%), the platelet level was from 30 to 49 x 109/l. In 43 patients (14.4%), platelet levels ranged from 50 to 99 x 109/L. In 2 patients (0.7%), the platelet count was greater than 100 x 109 / the median platelet level before the start of GCS was 18 x 109 / L. (p<0.0001). After 6 weeks of prednisolone therapy, platelet levels were: 0 to 29 x 109/l in 82 (27.5%) patients, low platelet levels persisted. Platelet levels from 30 to 49 x 109/l in 38 patients 12.8%. Platelet levels from 50 to 99 in x 109/l were achieved in 87 patients - 29.2%. 92 patients - 30.5%. The median platelet level after 6 weeks of taking GCS was 64 x 109/l (p<0.0001). Despite huge advances in the treatment of immune thrombocytopenia, glucocorticosteroids (prednisolone or dexamethasone) remain the drug of choice in the first line of therapy. Treatment of this group of drugs allows in most cases to achieve an optimal level of platelets and prevent bleeding episodes in most cases. Corticosteroids have firmly taken a leading position worldwide as the gold standard for the treatment of patients with immune thrombocytopenia in the first line of therapy.


Key words

orphan disease, platelet, immune thrombocytopenia, hemorrhagic syndrome, first line therapy, corticosteroids, complete response.





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