Journal Article

SIL-TAL1 Rearrangement is Related with Poor Outcome: A Study from a Chinese Institution

Huang, Liang, Xiong, Jie, Xu, Danmei, Wang, Di, He, Jing, Zhou, Xiaoxi, Zhu, Guangrong, Zhou, Shiqiu, Yang, Yunfan, Zhou, Jianfeng, Li, Chunrui, Wang, Na, Jiang, Lijun

2013

Overview

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.

• Publisher: Public Library of Science,Public Library of Science (PLoS)
• Subject: Acute lymphoblastic leukemia / Adolescent / Adult / Adults / Analysis / Animal models / Animals / Blood / Bone marrow / Cell survival / Chemotherapy / Child / Child, Preschool / Coagulation / Computer simulation / Cortex / Disease Models, Animal / Disseminated intravascular coagulation / Disseminated Intravascular Coagulation - etiology / Female / Health aspects / Hematology / Hospitals / Humans / Leukemia / Leukocytes / Lymphatic leukemia / Lymphocytes T / Lymphoma / Lysis / Male / Medical prognosis / Mice / Middle Aged / Oncogene Proteins, Fusion - genetics / Patient Outcome Assessment / Patients / Peripheral blood / Phenotype / Precursor T-Cell Lymphoblastic Leukemia-Lymphoma - complications / Precursor T-Cell Lymphoblastic Leukemia-Lymphoma - drug therapy / Precursor T-Cell Lymphoblastic Leukemia-Lymphoma - genetics / Precursor T-Cell Lymphoblastic Leukemia-Lymphoma - mortality / Prognosis / Retrospective Studies / RNA-Binding Protein FUS - genetics / RNA-Binding Protein FUS - metabolism / Science / Survival / T cells / Translocation, Genetic / Tumor Lysis Syndrome - etiology / White blood cell count / Xenograft Model Antitumor Assays / Xenografts / Xenotransplantation / Young Adult