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  • 1
    In: Blood, American Society of Hematology, Vol. 128, No. 22 ( 2016-12-02), p. 4941-4941
    Abstract: Introduction. Primary immune thrombocytopenia is a rare disease1. The incidence of ITP is not well estimated in Russia and worldwide. In adults it varies from 1,6 to 3,9/100 000 person-years2-3. The gender and age-associated results are discussed and differ in several investigations4-6. Study objectives: evaluation of the incidence of primary immune thrombocytopenia in adults in one region of Russia Patients and methods. The data source is the Registry of the patients with primary ITP in Russia. 272 adult patients: 77 males (28%) and 195 females (72%), age from 16 to 89 years (median 44 years) with ITP (ICD-10 code D69.3), newly diagnosed cases during the period from 12 Jan 2014 to 24 May 2016. Results. 221 (81%) cases were newly diagnosed in 12 regions of Russia in which registration was performed most actively - more than 5 cases for the duration of the study. But only one region was selected for the first evaluation of epidemiological characteristics because of the number of reasons. There is one hematological central clinic in this region in which diagnosis of ITP can be verified and patients with ITP are treated and monitored most properly. The early started and fully performed registration process can be regarded as covered most part of region population in this target region. 86 cases (27 male, 59 female) were registered in the target region. The gender-age distribution was following: male: age 〈 41 = 10 (37%), age 〈 41-60 = 7 (26%), age 〉 60 = 10 (37%); female: age 〈 29 = 10 (49%), age 〈 41-60 = 15 (25%), age 〉 60 = 15 (25%). The estimated incidence rate in the target region is shown in table 1. The estimated incidence rates in gender-age strata in the target region are demonstrated in table 2. Conclusion. Overall ITP incidence in one region of Russia is 3.20/100 000 person-years. It is compatible to the incidence in other European countries. Our data demonstrate the rise of incidence rate in males with age and its decrease with age in female population. Literature. 1) Rodeghiero F., Stasi R., Gernsheimer T., Michel M., Provan D., Arnold D.M., et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from international working group. Blood. 2009; 113(11): 2386--93. doi: 10.1182/blood-2008-07-162503. 2) Terrell DR, Beebe LA, Vesely SK, Neas BR, Segal JB, George JN. The incidence of immune thrombocytopenic purpura in children and adults: A critical review of published reports. Am J Hematol. 2010; 85(3): 174-180. 3) Moulis G, Palmaro A, Montastruc J-L, Godeau B, Lapeyre-Mestre M, Sailler L. Epidemiology of incident immune thrombocytopenia: a natiowide population-based study in France. Blood. 2014; 124(22): 3308-3315. 4) Segal JB, Powe NR. Prevalence of immune thrombocytopenia: analyses of administrative data. J Thromb Haemost 2006; 4: 2377-83 5) Schoonen WM, Kucera G, Coelson J, et al. Epidemiology of immune thrombocytopenic purpura in the General Practise Research Database. Br J Haematol 2009; 145(2): 235-244. 6) Lisukov I.A., Maschan A.A., Shamardina A.V., Chagorova T.V., Davydkin I.L., Sycheva T.M., et al. Immune thrombocytopenia: clinical manifestations and response to therapy. Intermediate analysis of data of the Russian register of patients with primary immune thrombocytopenia and review of literature. Oncogematologiya. 2013; 2: 61--9]. Disclosures No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
    RVK:
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2016
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 2
    In: Blood, American Society of Hematology, Vol. 132, No. Supplement 1 ( 2018-11-29), p. 5185-5185
    Abstract: Introduction RALL-2009 study (NCT01193933) has demonstrated that non-intensive but non-interruptive treatment with fewer allo-HSCT is rather effective in adult Ph-negative ALL pts aged 18-55 yy, producing more than 50% OS at 8-years [Parovichnikova, EHA E836, 2017]. In this study we have shown that only age, initial WBC 〉 30*109/l and t(4;11) became the factors of poor prognosis for BCP-ALL and none of the factors - for T-ALL. MRD was not measured in this study. Since Dec 2016 we started a new RALL-2016 (NCT03462095) protocol based on the same principle but modified according to the conclusions drawn from RALL-2009. Aim. To evaluate the first interim results of MRD monitoring and 1-year probability of relapse regarding MRD status in Ph-negative ALL treated by RALL-2016 protocol. Materials and patients. Taking in consideration the major pitfalls of RALL-2009 (high CR rate, early CNS relapses in T-ALL, selection bias for autologous HSCT in T-cell ALL, absence of MRD testing) a new study was developed. One day high-dose MTX block and one-day high-dose ARA-C block are eliminated and substituted by 2 months of non-intensive and non-interruptive treatment, L-asparaginase is scheduled for 1 year of treatment instead of 2,5 y, 15 intrathecal injections are increased up to 21 during consolidation phase, CR T-ALL patients are brought to randomization after the informed consent: auto-HSCT vs no auto-HSCT, - with further similar maintenance. All primary bone marrow samples are collected and tested for cytogenetic and molecular markers, all included pts are MRD monitored by flow cytometry in a centralized lab at 3-time points (days +70,+133,+190). Since Dec2016 till July 2018, 86 adult Ph-negative ALL pts from 11 centers (10 regions of Russia) were included in theRALL-2016 protocol: median age 33 y (18-54), m/f 54/32, BCP-ALL was diagnosed in 48 (56%), T-ALL/LBL - in 35 (40,5%), biphenotypic ALL -3 (3,5%). Results. CR rate in 76 available for the analysis patients was 80% (n=61), induction death occurred in 12% (n=9) and refractory ALL was registered in 8% (n=6). There were no deaths in CR so far. 2 allo-HSCT were performed (1 MUD and 1 haplo) for BCP-ALL with MRD persistence and T-ALL associated with Nijmegen breakage syndrome, respectively. 26 T-ALL patients after CR achievement were randomized for chemo (n=13) or for auto-HSCT (n=13). Up to now 7 of randomized T-ALL patients were transplanted at a median of 6 mo of CR. OS for the whole cohort constituted 68% at 18 months, relapse probability - 8,7%. MRD at the 1st time point (+70 day) was measured in 54 pts, at the 2nd time point (+133 day) - in 43 pts and at the 3rd time point (+190 day) - in 36 pts. MRD-positivity was detected in 15 pts (28%) at day+70 (BCP-ALL=11 out of 32 pts, T-ALL=4 out of 22), at day +133 - in 8 pts (19%) (BCP-ALL=7 out of 30 pts, T-ALL=1 out of 13), at day +190 - in 2 pts (5%) (both BCP-ALL). MRD clearance was much better in T-ALL patients, as it was demonstrated by other studies earlier [Bruggemen, Goekbuget]. But we have to mention that regardless our non-intensive approach, the portion of MRD-positive patients was similar at the same time points as in the other studies applying highly intensive protocol. We did not reveal any differences in early (within 1-year) relapse probability according to MRD status, though we have to assume that the study is small and the period of follow is too short. Conclusion Our data demonstrate that non-intensive but non-interruptive approach is as effective as more intensive protocols providing very similar MRD clearance in Ph-negative ALL. MRD is declining better in T-ALL patients comparing to BCP-ALL. And no correspondence was noticed between the MRD-positivity and relapse probability at the 18 mo of follow-up. Figure. Figure. Disclosures Kulikov: Russian Foundation for Basic Research grant 18-015-00399 A: Research Funding.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
    RVK:
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2018
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 3
    In: Journal of Siberian Federal University. Biology, Siberian Federal University, Vol. 4, No. 3 ( 2011-09), p. 281-292
    Type of Medium: Online Resource
    ISSN: 1997-1389 , 2313-5530
    Language: Unknown
    Publisher: Siberian Federal University
    Publication Date: 2011
    detail.hit.zdb_id: 2925361-5
    SSG: 12
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