[Immunosupressive therapy of aplastic anemia patients: successes and failures (single center experiment 2007-2016)].

Abstract:

:Treatment programs for patients with acquired aplastic anemia include two main therapeutic options: allogeneic bone marrow transplantation and combined immunosuppressive therapy (IST). However, combined IST remains the method of choice for most adult AA patients. This study included 120 AA patients who received IST at the National Research Center for Hematology in 20072016. The analysis was applied to 120 patients. Median age was 25 (1765) years, M/F: 66/54, SAA/NSAA: 66%/34%. Effectiveness of IST was carried out in 120 patients with AA. This group did not include 8 SAA patients who died during the first 3 months from the start of treatment from severe infectious complications (early deaths 6.2%) and 2 AA patients who dropped out of surveillance. The observation time was 55 (6120) months. Paroxysmal nocturnal hemoglobinuria (PNH clone) was detected in 67% of AA patients. The median PNH clone size (granulocytes) was 2.5 (0.0199.5)%. The treatment was according to the classical protocol of combined IST: horse antithymocytic globulin and cyclosporin A. Most of patients (87%) responded to combined immunosuppressive therapy. To achieve a positive response, it was sufficient to conduct one course of ATG to 64% of patients, two courses of ATG 24% of patients and 2% of patients responded only after the third course of ATG. A positive response after the first course was obtained in 64% of patients included in the analysis. Most of the responding patients (93%) achieve a positive response after 36 months from the start of treatment. Therefore, the 3rd6th months after the first course of ATG in the absence of an answer to the first line of therapy can be considered the optimal time for the second course of ATG. This tactic allows to get an answer in another 58% of patients who did not respond to the first course of ATG. The probability of an overall 10-year survival rate was 90% (95% confidence interval 83.696.2). :Современные программы лечения больных апластической анемией (АА) включают два основных терапевтических направления: трансплантацию аллогенного костного мозга и комбинированную иммуносупрессивную терапию (ИСТ). Методом выбора для большинства взрослых больных АА остается комбинированная ИСТ. В данную работу первоначально включены 130 пациентов с АА, получавших ИСТ в ФГБУ НМИЦ гематологии в 20072016 гг., анализ эффективности ИСТ проводился у 120 больных. Медиана возраста составила 25 (1765) лет, 70 мужчин и 50 женщин, у 66% больных диагностирована тяжелая АА и у 34% нетяжелая. Анализ эффективности ИСТ выполнялся у 120 больных АА. В группу анализа не включены 8 больных тяжелой АА, умерших в течение первых 3 мес от начала лечения от тяжелых инфекционных осложнений (ранние смерти 6,2%), и 2 больных АА, течение заболевания которых неизвестно. Медиана продолжительности наблюдения 55 (6120) мес. Клон пароксизмальной ночной гемоглобинурии выявлен у 67% больных АА, медиана его размера по гранулоцитам 2,5 (0,0199,5)%. Лечение проводилось по классическому протоколу комбинированной ИСТ: лошадиный антитимоцитарный глобулин (АТГ) и циклоспорин А. У 87% больных достигнут положительный ответ на комбинированную ИСТ. Для получения положительного ответа оказалось достаточным проведение одного курса терапии АТГ 74% больных, 2 курсов АТГ 24%, и 2% потребовалось 3 курса АТГ. У большинства больных (93%) гематологический ответ получен через 36 мес от начала лечения. Поэтому 36-й месяцы после 1-го курса АТГ при отсутствии ответа на 1-ю линию терапии можно считать оптимальным сроком проведения 2-го курса АТГ. Такая тактика позволяет получить ответ еще у 58% больных, не ответивших на 1-й курс терапии АТГ. Вероятность 10-летней общей выживаемости составила 90% (95% доверительный интервал 83,696,2).

journal_name

Ter Arkh

journal_title

Terapevticheskii arkhiv

authors

Mikhaylova EA,Fidarova ZT,Abramova AV,Luchkin AV,Troitskaya VV,Dvirnyk VN,Galtseva IV,Kliasova GA,Kovrigina AM,Kulikov SM,Chabaeva YА,Parovichnikova EN,Savchenko VG,Obukhova TN

doi

10.26442/00403660.2020.07.000756

subject

Has Abstract

pub_date

2020-09-01 00:00:00

pages

4-9

issue

7

eissn

0040-3660

issn

2309-5342

journal_volume

92

pub_type

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