DNA Index and the pretreatment proliferative activity of blastic cells were assessed in relation to treatment results in 58 children with acute lymphoblastic leukemia. ALL patients were treated with ...the use of BFM 79 protocol. After the median follow-up time of 32 months p-EFS and p-DFS were 0,425 and 0,495 respectively. There were 35 (56.45%) diploic, 21 (33.81%) hyper-diploic and 2 (3.22%) hypo-diploic patients. There was no statistical difference in p-EFS and p-DFS between diploic and hyper-diploic patients. Patients in I-st remission were characterized by significantly higher percentage of cells remaining in the S-phase of the cell cycle.
Introduction: Bone marrow transplantation (BMT) from HLA identical family donors is the treatment chosen for children with (SAA). When no donor is available, combined immunosuppressive therapy (IS) ...is given.
Material and method: SAA was recognised in 85 children (31 girls, 54 boys) aged 2–17.5 years in ten haematological clinic in Poland between 1993–2003 years. All patients received IS according SAA Working Party of the EBMT protocol: ALG or ATG, CsA, prenisolon and in 79 patients G-CSF or GM-CSF was additionally administered. Haematological response was evaluated on day 180 of therapy.
Results and conclusion: Complete remission (CR) occurred in 43 patients (40.5%), partial remission (PR) on 22 (25.8%), no response (NR) was obtained in 20 children (23.7%). Period of observation was from 12 months to 10.5 years. During this time relapse occurred in 10 patients (8.5%). Patients with NR and relapse were treated second course of IS or BMT from matched bone marrow donors. Unrelated BMT was performed in 11 patients with NR after IS. We observed 17 deaths (10 early during the first 3 months of IS) in all group. At present 54 children (63.5%) is in first remission (CR or PR) with lasts from 12 months to 10 years. During the 10.5 year of observation we notice one case with late clonal complication (PNH). Transformation to neoplasma was not observed in none of our patients after IS.
Relationship between the result of therapy in 48 cases of the acute lymphoblastic leukemia in childhood and character of response to corticosteroids, classified according to BMF group, has been ...assessed. Follow up period ranged from 13 to 75 months (mean 36 months, median 39 months). In was found, that the probability of survival free from any events, probability of complete remission persistence, and probability of survival after diagnosis have been statistically significantly higher in the group of patients with positive response to corticosteroids in comparison with patients non-responding to these agents. However, there was no significant difference in the number of recurrencies with the involvement of CNS. Authors share the opinion that their results confirm an opinion of Riehm et al. that the response to corticosteroids is of prognostic value in the acute lymphoblastic leukemia in childhood.
Background: From 1983, standardized therapeutic protocols for pediatric acute myeloid leukemia (AML) based on the BFM group experience were introduced in Poland. We retrospectively analyzed the ...results of pediatric AML treatment in Poland from 1983 to 2019 (excluding promyelocytic, therapy-related, biphenotypic, and Down syndrome AML). Methods: The study included 899 children suffering from AML treated with the following: AML-PPPLBC 83 (1983–1993, n = 187), AML-PPGLBC 94 (1994–1997, n = 74), AML-PPGLBC 98 (1998–2004, n = 151), AML-BFM 2004 Interim (2004–2015, n = 356), and AML-BFM 2012 (2015–2019, n = 131). Results: The probability of three-year overall survival was 0.34 ± 0.03, 0.37 ± 0.05, 0.54 ± 0.04, 0.67 ± 0.03, and 0.75 ± 0.05; event-free survival was 0.31 ± 0.03, 0.34 ± 0.05, 0.44 ± 0.04, 0.53 ± 0.03, and 0.67 ± 0.05; and relapse-free survival was 0.52 ± 0.03, 0.65 ± 0.05, 0.58 ± 0.04, 0.66 ± 0.03, and 0.78 ± 0.05, respectively, in the subsequent periods. A systematic reduction of early deaths and deaths in remission was achieved, while the percentage of relapses decreased only in the last therapeutic period. Surprisingly good results were obtained in the group of patients treated with AML-BFM 2012 with unfavorable genetic abnormalities like KMT2A-MLLT10/t(10;11)(p12;q23) and DEK-NUP214/t(6;9)(p23;q24), while unsatisfactory outcomes were found in the patients with FLT3-ITD. Conclusions: The use of standardized, systematically modified therapeutic protocols, with the successive consideration of genetic prognostic factors, and advances in supportive care led to a significant improvement in AML treatment outcomes over the last 40 years.
Background: The FMS-like tyrosine kinase 3 (FLT3) gene mutated in 10–15% of pediatric acute myeloid leukemia (AML) is associated with an inferior outcome. The aim of the study was to analyze the ...outcome and characteristics of FLT3-ITD-positive pediatric AML. Methods: We retrospectively analyzed the nationwide pediatric AML database from between 2005 and 2022. FLT3-ITD was found in 54/497 (10.7%) patients with available analysis. Three consecutive treatment protocols were used (AML-BFM 2004 Interim, AML-BFM 2012 Registry, AML-BFM 2019 recommendations). Results: Probabilities of 5-year overall (OS), event-free (EFS) and relapse-free survival were significantly lower in the FLT3-ITD-positive patients compared to FLT3-ITD-negative (0.54 vs. 0.71, p = 0.041; 0.36 vs. 0.59, p = 0.0004; 0.47 vs. 0.70, p = 0.0029, accordingly). An improvement in the outcome was found in the analyzed period of time, with a trend of better survival in patients treated under the AML-BFM 2012 and AML-BFM 2019 protocols compared to the AML-BFM 2004 protocol (5-year EFS 0.52 vs. 0.27, p = 0.069). There was a trend of improved outcomes in patients treated with FLT3 inhibitors (n = 9, 2-year EFS 0.67 vs. 0.33, p = 0.053) and those who received stem cell transplantation (SCT) (n = 26; 5-year EFS 0.70 vs. 0.27, p = 0.059). The co-occurrence of the WT1 mutation had a dismal impact on the prognosis (5-year EFS 0.23 vs. 0.69, p = 0.002), while the NPM1 mutation improved survival (5-year OS 1.0 vs. 0.44, p = 0.036). Conclusions: It seems that SCT and FLT3 inhibitors have a beneficial impact on the prognosis. Additional genetic alterations, like the WT1 and NPM1 mutations, significantly influence the outcome.
Acute P./myeloid leukemia post cytotoxic therapy (AML-pCT) is rare complication of cancer treatment in childhood. The objective of the study was to identify clinical characteristics and provide an ...analysis of the outcomes in pediatric AML-pCT. We retrospectively analyzed the data of 40 children with AML-pCT, treated from 2005 to 2020 within the Polish Pediatric Leukemia and Lymphoma Study Group. The most common primary malignancies were acute lymphoblastic leukemia (32.5%) and brain tumors (20%). The median latency period was 2.9 years (range: 0.7-12.9). Probabilities of overall (OS), event-free (EFS), and relapse-free survival (RFS) in the whole cohort were 0.49 ± 0.08, 0.43 ± 0.08, and 0.64 ± 0.10, respectively. Significant improvements in outcomes were observed in patients treated from 2015-2022 (two induction cycles followed by stem cell transplantation-SCT in 69% of patients) compared to 2005-2014 (four induction cycles followed by SCT in 49% of patients). The probability of EFS increased from 0.30 ± 0.10 to 0.67 ± 0.12 (
= 0.07) and RFS increased from 0.46 ± 0.11 to 1.0 (
= 0.01). The poorest outcome (OS and EFS 0.25 ± 0.20) was in AML post brain tumor, mainly due to deaths from toxicities. To conclude, treatment results achieved in patients with AML-pCT treated from 2015-2022, with two induction cycles followed by immediate SCT, were better than those reported by other authors, and comparable to the results in de novo AML.
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