| Objective: To investigate the clinical characteristics of T lymphoblastic lymphoma /leukemia, including sex, age, stage, ECOG score, proportion of mediastinal involvement,proportion of bone marrow involvement, immunophenotypic features, chromosome karyotype; To analyze the influence of various clinical features on prognosis and the effects of different treatment strategies including different chemotherapy regimens, allogeneic and autologous hematopoietic stem cell transplantation on prognosis; to assess the value of using the next generation sequencing and deacetylase inhibitor in refractory / relapsed patients.Methods: A total of 110 patients with confirmed T lymphoblastic lymphoma / leukemia were included in this study: 92 patients from the Department of Hematology of 301 Hospital between January 2000 to September 2016; 11 patients from the Department of Hematology of 307 Hospital between January 2010 to September 2015; 7 patients from the Department of Hematology of 304 Hospital between September 2009 to July 2015. Follow-up was conducted by telephone or outpatient referral. WBC, PLT count, LDH level, immunophenotypic characteristics and chromosome karyotype were analyzed. The relationship between the prognosis and the sex, age, stage, ECOG score, mediastinal involvement ratio, bone marrow involvement ratio were analyzed. Differences in the prognosis of different treatment methods were compared.Results: 1. Rate of complete remission in My- group was significantly higher than that in the My+ group (57% vs. 83%, P = 0.061); 2. 22 out of 43 (51.2%) bone marrow invovled patients were presented normal karyotype, and 21 cases (48.8%) contained abnormal chromosome karyotype, while the most common chromosome karyotype was t (10; 11); 3.35 cases (44.3%) had one or more gene abnormalities, such as TCR gene rearrangement,increased expression ofHOX11, BCL-2, c-myc and HOX11 / TCR fusion. 8 of 9 patients had one or more gene mutations by NGS; 4. Age over 50 years and non-CR1 were independent adverse prognostic factor; 5. Rate of complete remission was 70.2%. 5 years overall survival and progression-free survival were 29.4%±6.3% and 25%±6.1%,respecrately; 6. 5 years OS of ALL-like chemotherapy and CHOP-like chemotherapy were 0 and 26.9% (P=0.016), and PFS were 0 and 18.1% (P=0.013) ; 7. 11 patients received autologous transplantation, 7 of whom relapsed after transplantation; 8. Allogeneichematopoietic stem cell transplantation had better 5 years PFS than ALL-like chemotherapy consolidation in CR1, which was 66.4%±11.8% and 37.5%± 13.3% (P=0.045) respectively,and a tend to improved OS (53.1%± 14.6% vs. 43.6%±15%, P=0.596),5 years cumulative recurrence rates were 33.6% and61.6% respectively (P = 0.045); 9. 5 years OS and PFS of 39 patients received allogeneic hematopoietic stem cell transplantation were 38.3% and 54.2%with a transplant-related mortality of 20.3% (8/39). The PFS of CR1 patients before transplantation was superior to that of non-CR1 patients before transplantation, with PFS of 66.4% and 34.4% (P = 0.029), respectively. OS and PFS were not affected by different donors (P> 0.05); 10. There was no difference of 5 years OS and PFS between haploid group and HLA-matched group. The transplantation related mortality (TRM) of haploid transplantation is higher than that of HLA-matched transplantation group, which was 35%and 5.3%, respectively (P = 0.014); 11、Five refractory or relapsed patients received Chidamide along with chemotherapy, 3 of them received CR, and 2 patients had no response;12. The common recurrence site of 52 relapsed patients included bone marrow (48.1%),mediastinum (25%) and CNS (17.3%).Conclusion: 1. T-LBL / ALL usually occurs in young men, mostly diagnosed at III / IV stage.Mediastinal mass was majority of onset, about half of cases of bone marrow involvement.Patients older than 50 years were with poor prognosis, whereas staging, ECOG Score and LDH levels do not affect prognosis; 2. TDT positive is an important immune marker, while TDT negative can not rule out the diagnosis of the disease. Lymphoma cells are often associated with B and myeloid markers, and CR rate of myeloid antigen-positive patients may be lower than that of the negative group; 3. About half of patients were diagnosed with chromosome karyotype abnormalities, involving 11q23 chromosome translocation; 4. About 45% of patients presented abnormal gene, with recurrence including TCR rearrangement,increased expression of HOX11, BCL-2, c-myc and HOX11 / TCR fusion gene. Second-generation sequencing may provide the necessary information for the stratification of the risk and deepen the understanding of the disease; 5. Prognosis of ALL-like chemotherapy is significantly better than CHOP-like chemotherapy; 6. Allogeneic hematopoietic stem cell transplantation gained a better PFS than ALL-like chemotherapy in CR1; 7. The prognosis of achieving CR1 before allogeneic hematopoietic stem cell transplantation was significantly better than that of non-CR1 patients before transplantation; 8. Despite the higher transplant-related mortality, haploid transplantation can achieve similar OS and PFS compared to HLA-matched transplantation. |
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