Open AccessSIL-TAL1 Rearrangement is Related with Poor Outcome: A Study from a Chinese Institution
Author(s) -
Di Wang,
Guoding Zhu,
Na Wang,
Xiaoxi Zhou,
Yunfan Yang,
Shixiang Zhou,
Jie Xiong,
Jing He,
Lijun Jiang,
Chunrui Li,
Danmei Xu,
Liang Huang,
Jianfeng Zhou
Publication year - 2013
Publication title -
plos one
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.99
H-Index - 332
ISSN - 1932-6203
DOI - 10.1371/journal.pone.0073865
Subject(s) - immunophenotyping , medicine , white blood cell , population , oncology , leukemia , gastroenterology , immunology , flow cytometry , environmental health
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.