Prognostic value and outcome for acute lymphocytic leukemia in children with MLL rearrangement: a case-control study
Abstract Purpose To evaluate the prognostic factors and outcome for acute lymphoblastic leukemia (ALL) in children with MLL rearrangement (MLL-r). Methods A total of 124 pediatric patients who were diagnosed with ALL were classified into two groups based on the MLL-r status by using a retrospective...
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| Format: | Article |
| Language: | English |
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BMC
2022-12-01
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| Series: | BMC Cancer |
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| Online Access: | https://doi.org/10.1186/s12885-022-10378-w |
| _version_ | 1811207958979149824 |
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| author | Kun-yin Qiu Dun-hua Zhou Xiong-yu Liao Ke Huang Yang Li Hong-gui Xu Wen-jun Weng Lu-hong Xu Jian-pei Fang |
| author_facet | Kun-yin Qiu Dun-hua Zhou Xiong-yu Liao Ke Huang Yang Li Hong-gui Xu Wen-jun Weng Lu-hong Xu Jian-pei Fang |
| author_sort | Kun-yin Qiu |
| collection | DOAJ |
| description | Abstract Purpose To evaluate the prognostic factors and outcome for acute lymphoblastic leukemia (ALL) in children with MLL rearrangement (MLL-r). Methods A total of 124 pediatric patients who were diagnosed with ALL were classified into two groups based on the MLL-r status by using a retrospective case-control study method from June 2008 to June 2020. Results The prevalence of MLL-r positive in the whole cohort was 4.9%. The complete remission (CR) rate on Day 33 in the MLL-r positive group was not statistically different from the negative group (96.8% vs 97.8%, P = 0.736). Multivariate analysis showed that T-cell, white blood cell counts (WBC) ≥ 50 × 109/L, MLL-AF4, and D15 minimal residual disease (MRD) positive were independent risk factors affecting the prognosis of MLL-r positive children. Stem cell transplantation (SCT) was a favorable independent prognostic factor affecting event-free survival (EFS) in MLL-r positive patients (P = 0.027), and there was a trend toward an independent prognostic effect on overall survival (OS) (P = 0.065). The 10-year predicted EFS for patients with MLL-AF4, MLL-PTD, MLL-ENL, other MLL partner genes, and MLL-r negative cases were 46.67 ± 28.61%, 85.71 ± 22.37%, 75 ± 32.41%, 75 ± 32.41%, and 77.33 ± 10.81%, respectively (P = 0.048). The 10-year predicted OS were 46.67 ± 28.61%, 85.71 ± 22.37%, 75 ± 32.41%, 75 ± 32.41%, and 85.2 ± 9.77%, respectively (P = 0.049). The 124 patients with ALL were followed up and eventually 5 (4%) cases relapsed, with a median relapse time of 3.9 years. Conclusion Patients with MLL-r positive ALL have moderate remission rates, but are prone to relapse with low overall survival. The outcome of MLL-r positive ALL was closely related to the partner genes, and clinical attention should be paid to screening for MLL partner genes and combining them with other prognostic factors for accurate risk stratification. |
| first_indexed | 2024-04-12T04:13:53Z |
| format | Article |
| id | doaj.art-05242078cda84627a5242240d75eeb76 |
| institution | Directory Open Access Journal |
| issn | 1471-2407 |
| language | English |
| last_indexed | 2024-04-12T04:13:53Z |
| publishDate | 2022-12-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Cancer |
| spelling | doaj.art-05242078cda84627a5242240d75eeb762022-12-22T03:48:27ZengBMCBMC Cancer1471-24072022-12-0122111310.1186/s12885-022-10378-wPrognostic value and outcome for acute lymphocytic leukemia in children with MLL rearrangement: a case-control studyKun-yin Qiu0Dun-hua Zhou1Xiong-yu Liao2Ke Huang3Yang Li4Hong-gui Xu5Wen-jun Weng6Lu-hong Xu7Jian-pei Fang8Children’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen UniversityChildren’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen UniversityChildren’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen UniversityChildren’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen UniversityChildren’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen UniversityChildren’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen UniversityChildren’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen UniversityChildren’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen UniversityChildren’s Medical Center, Sun Yat-sen Memorial Hospital, Sun Yat-sen UniversityAbstract Purpose To evaluate the prognostic factors and outcome for acute lymphoblastic leukemia (ALL) in children with MLL rearrangement (MLL-r). Methods A total of 124 pediatric patients who were diagnosed with ALL were classified into two groups based on the MLL-r status by using a retrospective case-control study method from June 2008 to June 2020. Results The prevalence of MLL-r positive in the whole cohort was 4.9%. The complete remission (CR) rate on Day 33 in the MLL-r positive group was not statistically different from the negative group (96.8% vs 97.8%, P = 0.736). Multivariate analysis showed that T-cell, white blood cell counts (WBC) ≥ 50 × 109/L, MLL-AF4, and D15 minimal residual disease (MRD) positive were independent risk factors affecting the prognosis of MLL-r positive children. Stem cell transplantation (SCT) was a favorable independent prognostic factor affecting event-free survival (EFS) in MLL-r positive patients (P = 0.027), and there was a trend toward an independent prognostic effect on overall survival (OS) (P = 0.065). The 10-year predicted EFS for patients with MLL-AF4, MLL-PTD, MLL-ENL, other MLL partner genes, and MLL-r negative cases were 46.67 ± 28.61%, 85.71 ± 22.37%, 75 ± 32.41%, 75 ± 32.41%, and 77.33 ± 10.81%, respectively (P = 0.048). The 10-year predicted OS were 46.67 ± 28.61%, 85.71 ± 22.37%, 75 ± 32.41%, 75 ± 32.41%, and 85.2 ± 9.77%, respectively (P = 0.049). The 124 patients with ALL were followed up and eventually 5 (4%) cases relapsed, with a median relapse time of 3.9 years. Conclusion Patients with MLL-r positive ALL have moderate remission rates, but are prone to relapse with low overall survival. The outcome of MLL-r positive ALL was closely related to the partner genes, and clinical attention should be paid to screening for MLL partner genes and combining them with other prognostic factors for accurate risk stratification.https://doi.org/10.1186/s12885-022-10378-wMLLChildrenAcute lymphoblastic leukemiaOutcomePrognosis |
| spellingShingle | Kun-yin Qiu Dun-hua Zhou Xiong-yu Liao Ke Huang Yang Li Hong-gui Xu Wen-jun Weng Lu-hong Xu Jian-pei Fang Prognostic value and outcome for acute lymphocytic leukemia in children with MLL rearrangement: a case-control study BMC Cancer MLL Children Acute lymphoblastic leukemia Outcome Prognosis |
| title | Prognostic value and outcome for acute lymphocytic leukemia in children with MLL rearrangement: a case-control study |
| title_full | Prognostic value and outcome for acute lymphocytic leukemia in children with MLL rearrangement: a case-control study |
| title_fullStr | Prognostic value and outcome for acute lymphocytic leukemia in children with MLL rearrangement: a case-control study |
| title_full_unstemmed | Prognostic value and outcome for acute lymphocytic leukemia in children with MLL rearrangement: a case-control study |
| title_short | Prognostic value and outcome for acute lymphocytic leukemia in children with MLL rearrangement: a case-control study |
| title_sort | prognostic value and outcome for acute lymphocytic leukemia in children with mll rearrangement a case control study |
| topic | MLL Children Acute lymphoblastic leukemia Outcome Prognosis |
| url | https://doi.org/10.1186/s12885-022-10378-w |
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