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  • 1
    Publication Date: 2014-11-07
    Description: Conventional myeloablative conditioning (MAC) regimens often cause severe regimen-related toxicity (RRT). Furthermore, many patients suffer from poor quality of life in accordance with the increase in long-term survivors. We therefore devised a reduced-toxicity myeloablative conditioning (RTMAC) regimen consisting of 8-Gy total body irradiation (TBI), fludarabine (FLU) and cyclophosphamide (CY) for pediatric hematological malignancies. A retrospective single-center analysis was performed on patients with leukemia or myelodysplastic syndrome (MDS), aged ≤20 years, who had received an 8-Gy TBI/FLU/CY RTMAC regimen followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT). Thirty-one patients underwent first allo-HSCT after an RTMAC regimen. The diagnoses were acute lymphoblastic leukemia (n = 11), acute myeloid leukemia (n = 13), MDS (n = 4), juvenile myelomonocytic leukemia (n = 1) and acute leukemias of ambiguous lineage (n = 2). While 3 patients showed early hematological relapse, the remaining 28 patients achieved engraftments. None of the patients developed grade 4 or 5 toxicities during the study period. The 5-year overall survival and relapse-free survival were 80% [95% confidence interval: CI, 61–91%] and 71% [95% CI, 52–84%], respectively. Our RTMAC regimen would be less toxic and offers a high probability of survival for children with hematological malignancies. Scientific Reports 4 doi: 10.1038/srep06942
    Electronic ISSN: 2045-2322
    Topics: Natural Sciences in General
    Published by Springer Nature
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  • 2
    Publication Date: 2005-11-16
    Description: The notch family of transmembrane receptors play crucial roles in cell fate determination through interaction with the Delta/Serrate /Lag-2 family of ligands. The transcription of the hairyl enhancer of split (HES-1) gene is one of the known target of notch signaling. Notch1 is involved in the pathogenesis of T-acute lymphoblastic leukemia (T-ALL) carrying the very rare translocation t (7;9)(q34;q34.3). However, the function of Notch signaling in leukemogenesis is currently still unclear. To investigate the possible role of notch signaling in human acute leukemia,A quantitative real-time reverse transcriptase polymerase chain reaction (RQ-RT-PCR) method has been established for detecting expression levels of Notch1~2, Jagged1, Delta1, Hes1 and internal reference GAPDH in primary BM samples of 34 acute leukemia patients and 11 normal controls by LightCycler using TaqMan probe; 34 pretreated acute leukemia patients includes 16 acute lymphoblastic leukemia (7 T-ALL, 9 B-ALL) and 18 acute myeloblastic leukemia (10 M1,3 M2,3 M3 and 2 M5) In all of these cases the leukemia blast cells are more than 80%. The results demonstrated as the follows: The average expression levels of Notch1 in 16 ALL were statistically higher than that in 11 normal and 18 AML [ 13225.0±3306.3 (16) vs 1725.6±1237.8 (11) vs 2354.8±2985.6(18), F=7.85, P0.05); the average expression levels of Notch2 did not have statistically difference among ALL, normal and AML.[143671.7±372814.9 (16) vs 52178.2±36747.5 (11) vs 66520.2±62643.2 (18) F=0.15, P〉0.05 ]. The average expression levels of Jagged1 in 16 ALL were statistically less than that in 18 AML and 11 normal [129.9±353.8(16) vs 375.4±697.5(18) vs 2212.5±3371.7(11), F=20.23, P
    Print ISSN: 0006-4971
    Electronic ISSN: 1528-0020
    Topics: Biology , Medicine
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