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Острые лейкозы со смешанным фенотипом: клинико-лабораторные особенности и прогнозстатья
Статья опубликована в журнале из списка Web of Science и/или Scopus
- Авторы: Антипова А.С., Баранова О.Ю., Френкель М.А., Ширин А.Д.
- Журнал: Клиническая онкогематология. Фундаментальные исследования и клиническая практика
- Том: 9
- Номер: 3
- Год издания: 2016
- Первая страница: 337
- Последняя страница: 338
- Аннотация: Background. Mixed phenotype acute leukemia (MPAL) is a rare type of acute leukemia. It includes biclonal and bipheno-typic AL which are not considered separate entities. Aim. To determine the clinical and laboratory features and prognosis in MPAL patients. Methods. Among 161 primary AL patients treated in the N.N. Blokhin Russian Cancer Research Center over the period from 2000 to 2014, MPAL was diagnosed in 5 cases (3.1 %). In total, the MPAL group consisted of 13 patients enrolled in this study. Only 5 of them were treated in the N.N. Blokhin Russian Cancer Research Center, whereas the remaining 8 patients were in four other hematology departments in Moscow. The disease was diagnosed according to 2008 WHO criteria. The median age was 48 years (ranged from 20 to 75). Results. MPAL-specific clinical features were not found. Ex-tramedullary lesions were very rare. Мyeloid/В-phenotype was found in most patients (n=11), and М/Т was found only in 2 patients. The CD34 antigen expression was observed almost in all patients (12 of 13) and was about 75.2 %. t(9;22)(q34;q11)/ BCR-ABL were detected by cytogenetic/molecular genetic PCR in 8 of 9 patients. Therefore, Ph+ MPAL was diagnosed in 9 patients. р190 and р210 transcripts were found in equal proportions. Treatment strategy was determined by molecular-cytogenetic profile of the disease. In the Ph+ MPAL group, patients received imatinib in combination with polychemotherapy regimens for acute lymphocytic leukemia (ALL) (n = 8). In the Ph- MPAL group, regimens for AML and regimens with cytostatic agents prescribed for both AML and ALL were used to induce remission (n = 4). The rate of complete remissions was 66.7 %, and the early mortality rate was 8.3 %. However, long-term results of therapy were unsatisfactory: 3-year overall survival was 20.4 % (median 28 months), 3-year relapse-free survival was 15 % (median 16 months). Complete remission was induced in all eight Ph+ MPAL patients. Conclusion. In Ph+ MPAL, the use of the 1st or 2nd generation tyrokinase inhibitors (TKI) is the obligatory part of the therapy. A combination of TKI and ALL-regimens seems more promising. The monitoring of the molecular and cytogenetic responses to TKI therapy, as well as the allo-HSCT in post consolidation phase also plays an important role in the treatment. The problem of the treatment of Ph- MPAL patients remains unresolved. Further studies of the molecular cytogenetic profile of this AL category are required for elaboration of optimal treatment regimens. Conflict of interest.
- Добавил в систему: Ширин Антон Дмитриевич