title: SIL-TAL1 rearrangement is related with poor outcome: a study from a Chinese institution.
abstract: SIL-TAL1 rearrangement is common in T-cell acute lymphoblastic leukemia (T-ALL), however its prognostic implication remains controversial. To investigate the clinical characteristics and outcome of this subtype in Chinese population, we systemically reviewed 62 patients with newly diagnosed T-ALL, including 15 patients with SIL-TAL1 rearrangement. We found that SIL-TAL1(+) T-ALL was characterized by higher white blood cell count (P = 0.029) at diagnosis, predominant cortical T-ALL immunophenotype (P = 0.028) of the leukemic blasts, and a higher prevalence of tumor lysis syndrome (TLS, P<0.001) and disseminated intravascular coagulation (DIC, P<0.001), which led to a higher early mortality (P = 0.011). Compared with SIL-TAL1(-) patients, SIL-TAL1(+) patients had shorter relapse free survival (P = 0.007) and overall survival (P = 0.002). Our NOD/SCID xenotransplantation model also demonstrated that SIL-TAL1(+) mice models had earlier disease onset, higher leukemia cell load in peripheral blood and shorter overall survival (P<0.001). Moreover, the SIL-TAL1(+) mice models exerted a tendency of TLS/DIC and seemed vulnerable towards chemotherapy, which further simulated our clinical settings. These data demonstrate that SIL-TAL1 rearrangement identifies a distinct subtype with inferior outcome which could allow for individual therapeutic stratification for T-ALL patients.
authors.affiliation: Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, The People's Republic of China.
authors.forename: Di
authors.initials: D
authors.lastname: Wang
authors.forename: Guangrong
authors.initials: G
authors.lastname: Zhu
authors.forename: Na
authors.initials: N
authors.lastname: Wang
authors.forename: Xiaoxi
authors.initials: X
authors.lastname: Zhou
authors.forename: Yunfan
authors.initials: Y
authors.lastname: Yang
authors.forename: Shiqiu
authors.initials: S
authors.lastname: Zhou
authors.forename: Jie
authors.initials: J
authors.lastname: Xiong
authors.forename: Jing
authors.initials: J
authors.lastname: He
authors.forename: Lijun
authors.initials: L
authors.lastname: Jiang
authors.forename: Chunrui
authors.initials: C
authors.lastname: Li
authors.forename: Danmei
authors.initials: D
authors.lastname: Xu
authors.forename: Liang
authors.initials: L
authors.lastname: Huang
authors.forename: Jianfeng
authors.initials: J
authors.lastname: Zhou
mesh_terms: D000293:Adolescent; D000328:Adult; D000818:Animals; D002648:Child; D002675:Child, Preschool; D004195:Disease Models, Animal; D004211:Disseminated Intravascular Coagulation / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D051379:Mice; D008875:Middle Aged; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D063868:Patient Outcome Assessment; D010641:Phenotype; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications / Q000188:drug therapy / Q000235:genetics / Q000401:mortality; D011379:Prognosis; D034702:RNA-Binding Protein FUS / Q000235:genetics / Q000378:metabolism; D012189:Retrospective Studies; D014178:Translocation, Genetic; D015275:Tumor Lysis Syndrome / Q000209:etiology; D023041:Xenograft Model Antitumor Assays; D055815:Young Adult
chemical_list: D015514:Oncogene Proteins, Fusion; D034702:RNA-Binding Protein FUS; C547584:SIL-TAL1 fusion protein, human
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