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Objectives: To investigate the prognosis of childhood T-lymphoblastic lymphoma (T-LBL) treated with acute lymphoblastic leukemia (ALL) regimen and related influencing factors., Methods: A retrospective analysis was performed for the prognostic characteristics of 29 children with T-LBL who were treated with ALL regimen (ALL-2009 or CCCG-ALL-2015 regimen) from May 2010 to May 2022., Results: The 29 children with T-LBL had a 5-year overall survival (OS) rate of 84%±7% and an event-free survival (EFS) rate of 81%±8%. The children with B systemic symptoms (unexplained fever >38°C for more than 3 days; night sweats; weight loss >10% within 6 months) at initial diagnosis had a lower 5-year EFS rate compared to the children without B symptoms ( P <0.05). The children with platelet count >400×10 9 /L and involvement of both mediastinum and lymph nodes at initial diagnosis had lower 5-year OS rates ( P <0.05). There were no significant differences in 5-year OS and EFS rates between the children treated with CCCG-ALL-2015 regimen and those treated with ALL-2009 regimen ( P >0.05). Compared with the ALL-2009 regimen, the CCCG-ALL-2015 regimen reduced the frequency of high-dose methotrexate chemotherapy and the incidence rate of severe infections ( P <0.05)., Conclusions: The ALL regimen is safe and effective in children with T-LBL. Children with B systemic symptoms, platelet count >400×10 9 /L, and involvement of both mediastinum and lymph nodes at initial diagnosis tend to have a poor prognosis. Reduction in the frequency of high-dose methotrexate chemotherapy can reduce the incidence rate of severe infections, but it does not affect prognosis.

Objective: To evaluate the efficacy of the Chinese Children's Leukemia Group (CCLG) acute lymphoblastic leukemia (ALL) 2008 protocol (CCLG-ALL 2008) in the treatment of children's T-cell acute lymphoblastic leukemia (T-ALL). Methods: Clinical characteristics and outcomes of 84 newly diagnosed T-ALL children (63 males and 21 females) treated with CCLG-ALL 2008 protocol from April 2008 to April 2015 in the Department of Pediatric Hematology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences were analyzed retrospectively. Kaplan-Meier analysis was used to evaluate the overall survival (OS) and event free survival (EFS), and COX regression was used to evaluate the influencing factors of OS and EFS. Results: (1) Baseline data: 84 children were included, 56 cases (67%) of children were younger than 10 years old. Patients whose white blood cell count≥50×10(9)/L ranked 70% (59/84). Karyotype: 58% (49/84) with normal karyotype, 10% (8/84) with abnormality of chromosome 11, 8%(7/84) with abnormality of chromosome 9, 2%(2/84) with abnormality in both chromosome 11 and chromosome 9, 8% (7/84) with other complex karyotypes. Fusion gene: 33%(28/84) were SIL-TAL1 positive. The patients were grouped by CCLG-ALL 2008 risk score, 40% (34/84) were in the intermediate risk group and 60% (50/84) in the high risk group. (2) Treatment efficacy: 84 cases were followed up until May 30, 2018. The follow-up time was 42.0 (0.3-120.0) months. The sensitivity rate of prednisone treatment was 56% (47/84); the complete response (CR) rate after the induction therapy of vincristine+daunoblastina+L-asparaginase+dexamethasone (VDLD)(d 33) was 88% (74/84); the total CR rate after VDLD induction combined with cyclophosphamide+cytarabine+6-mercaptopurine (CAM) treatment (d80) was 94% (79/84); the recurrence rate was 24% (20/84). Among the 20 recurrent cases, there were 13 cases (65%) with ultra-early recurrence (within 18 months after diagnosis), 6 cases (30%) with early recurrence (18 to 36 months after diagnosis); 1 patient (5%) with late recurrence (over 36 months after diagnosis). During the follow-up period, twenty-eight children (33%) died (22 cases with recurrence or suspending treatment without remission, 2 cases with infection, 1 case of sudden death in chemotherapy, 1 patient failed in transplantation, 1 patient with severe cirrhosis, and 1 patient with unknown cause). (3) Kaplan-Meier analysis: the 5-year OS and EFS of the 84 children were (63±6)% and (60±6)% respectively. (4) Efficacy in different risk groups: prednisone sensitivity rates in the two different risk groups were 100% (34/34) and 26% (13/50), respectively (χ(2)=3.237, P< 0.05). The CR rates at the end of VDLD induction therapy (d 33) were 100% (34/34) and 80% (40/50), respectively (χ(2)=2.767, P< 0.05). The recurrence rate of children in the two groups was 12% (4/34) and 32% (16/50), respectively (χ(2)=4.245, P< 0.05).The mortality rates of the two groups were 21% (7/34) and 42% (21/50), respectively (χ(2)=3.198, P< 0.05). Kaplan-Meier analysis showed that the 5-year OS of the two groups were (77±7)% and (53±8)%; and the 5-year EFS of the two groups were (75±8)% and (49±8)% (χ(2)=4.235, 3.875, both P <0.05) . (5) COX multivariate regression analysis showed that the classification of risk according to CCLG-ALL 2008 was an important factor influencing the prognosis of children with T-ALL ( OR =3.313, 95% CI 1.165-9.422, P =0.025). Conclusions: The results of the risk group treatment according to the CCLG-ALL 2008 protocol showed that the long-term survival of children with middle risk was significantly better than that of children at high risk.

Objective: To study the expression of β-integrin family members in children with T-cell acute lymphoblastic leukemia (T-ALL) and their significance., Methods: Quantitative real-time PCR analyses were performed to assess the expression levels of β-integrin family members in bone marrow samples from 22 children with newly-diagnosed T-ALL and 21 controls (16 children with non-malignant hematologic disease and 5 healthy donors with bone marrow transplantation). Jurkat cells were treated with integrin inhibitor arginine-glycine-aspartate (Arg-Gly-Asp, RGD) peptide. The cell viability and apoptosis rate were determined by CCK8 assay and flow cytometry respectively., Results: The mRNA levels of integrins β 2 , β 3 , and β 5 were significantly lower in children with T-ALL than in controls (P<0.05). In T-ALL patients, high integrin β 3 expression was associated with lower white blood cell counts (<100×10 9 /L), minimal residual disease (MRD) positivity, and day 33 bone marrow negative remission (P<0.05). In T-ALL patients, higher integrin β 5 expression was associated with relapse of T-ALL (P<0.05). Based on survival curve analysis, higher integrin β 3 expression was related to lower event-free survival and overall survival rates. RGD peptide treatment inhibited the proliferation of Jurkat cells and increased their apoptosis rate (P<0.05)., Conclusions: β-Integrin may play a role in the occurrence and development of T-ALL by affecting cell proliferation and apoptosis. The expression of integrin β5 is closely related to the risk of relapse of T-ALL. The expression of integrin β3 is closely related the treatment response and prognosis of T-ALL.

This study was aimed to explore the clinical features and prognosis outcome of childhood T-cell acute lymphoblastic leukemia (T-ALL). The clinical data of 38 cases of newly diagnosed T-ALL from Jan 2005 to Aug 2010 were analyzed retrospectively, and 78 cases of B-ALL with intermediate and high risk were collected as control group, then the sensitive rate of patients to prednisone pretreatment, complete remission (CR) rate at day 33 after induction chemotherapy, relapse rate and 3-year event-free survival (EFS) were compared between T-ALL and B-ALL children. The results showed that no significant statistic difference were found in distribution of age, infiltration of liver, spleen and lymph nodes as well as central nervous system disease, chromosome abnormality, expression level of fusion gene and so on between T-ALL and B-ALL groups (p > 0.05), but there were significant differences in sex and number of cases with WBC count ≥ 50 × 10(9)/L between them (p < 0.05). The sensitive rate of T-ALL and B-ALL patients to prednisone pretreatment was 51.9% and 89.3% respectively (p < 0.05). The ratio failed to achieve CR at day 33 after induction chemotherapy was 15.4% and 8.1% in the two groups (p > 0.05). The relapse rate of T-ALL and B-ALL cases was 30.8% (8/26) and 14.9% (11/74) respectively (p > 0.05). The time from CR to relapse was (9.78 ± 3.48) month and (21.28 ± 14.32) month (p < 0.05). The 3 year EFS of T-ALL cases with intermediate and high risk was (37.5 ± 17.1)% and (22.2 ± 9.8)%, while 3 year EFS of B-ALL cases was (66.7 ± 7)% and (51.7 ± 9.3)% respectively (p < 0.05) according to Kaplan-Meier survival curve. It is concluded that as compared with B-ALL cases, the male ratio and initial WBC count are higher, moreover the early response to prednisone pretreatment and 3 year EFS are poor in T-ALL cases, the prognosis outcome is poor also.

Objective: Hematologic relapse remains the greatest obstacle to the cure of acute lymphoblastic leukemia (ALL), especially T-lineage acute lymphoblastic leukemia (T-ALL) in children. Recent studies have shown that patients with increased risk of relapse can be identified by measuring residual leukemic cells, called minimal residual disease (MRD), during clinical remission. Current polymerase chain reaction (PCR) methods, however, for measuring MRD are cumbersome and time-consuming. To improve and simplify MRD assessment, the author developed a real-time quantitative PCR (RQ-PCR) assay for the detection of leukemic cells that harbor the tal-1 deletion. In addition, the author discussed the significance of MRD levels at different stages in treatment and prognosis of children with T-ALL., Methods: A total of 50 consecutively enrolled patients with T-ALL were analysed for detection of leukemic cells harboring the most common tal-1 deletion. Serial dilutions of leukemic DNA were studied to find the sensitivity of detection with RQ-PCR assay. The MRD of 28 samples in clinical remission from 10 patients were quantified by RQ-PCR assay and limiting dilution assay. The results detected by both methods were compared statistically with correlation analysis., Results: (1) A total of 10 patients presented tal-1 deletion involving the sildb1 breakpoint rearranged to tal1db1 in 50 cases with T-ALL. The breakpoints of relapsed samples are the same as those of the corresponding diagnostic samples; (2) The RQ-PCR assay had a sensitivity of detection of one leukemic cell among 100,000 normal cells. In 24 samples, MRD levels > 10(-5) could be detected with both methods. The percentages of leukemic cells measured by the two methods correlated well (r = 0.898, P < 0.001); (3) The MRD levels of 3 patients out of the 8 cases undergoing disciplinary regimen were over 10(-4) at the end of induction chemotherapy. They all relapsed in bone marrow during chemotherapy. The higher the MRD levels, the earlier the relapse. The other 5 patients with MRD levels < 10(-4) had been relapse-free survival (RFS) for 4-59 months, one of whom with increased MRD levels > 10(-4) for twice at the continuation stage had been RFS for 27 months till now., Conclusions: The sildb1-taldb1 deletion presents in 20% of T-ALL, and is an ideal PCR marker for its specificity, uniform and stability; The tal-1 RQ-PCR can be used for the rapidly, sensitively and accurately quantitative assessment of MRD in T-ALL with the tal-1 deletion. MRD levels at different stages of chemotherapy have different significance in prognosis and treatment.