Department of Pediatric Hematology-Oncology, Children's Mercy Hospital, Kansas City, MO.
Stem Cell Biology Lab, Autonomous University of Morelos State, Cuernavaca.
J Pediatr Hematol Oncol. 2024 Mar 1;46(2):e131-e136. doi: 10.1097/MPH.0000000000002802. Epub 2023 Dec 22.
Mexican and Hispanic children in Mexico and the United States, respectively, have the highest incidence and worst outcomes of pre-B acute lymphoblastic leukemia (ALL) compared with other racial/ethnic groups. Terminal deoxynucleotidyl transferase (TdT) is an intranuclear DNA polymerase normally present on immature lymphocytes (TdT-positive) and distinguishes ALL from mature lymphoid malignancies. We performed a multisite retrospective study to determine the incidence of TdT-negative precursor B-cell acute lymphoblastic leukemia (pre-B ALL) among Mexican, Caucasian, and US-born Hispanic children to correlate TdT expression with patient characteristics and known prognostic factors. Fisher exact test was performed for categorical variables and the Wilcoxon rank-sum test was used for continuous variables. TdT-negative pre-B ALL was most frequently identified in patients with National Cancer Institute high-risk disease ( P =0.014). TdT-negative expression was also most frequently associated with hypodiploid pre-B ALL ( P =0.001) and KMT2A gene rearrangement ( P =0.0012). Mexican children had the highest incidence of TdT-negative ALL compared with Caucasians and US Hispanics ( P <0.001), with an increased incidence of poor prognostic features as well. This study demonstrates significant differences in TdT-negative expression, genomic alterations, and leukemic ploidy based on race and ethnicity.
墨西哥和美国的墨西哥裔和西班牙裔儿童,与其他种族/民族群体相比,前 B 急性淋巴细胞白血病 (ALL) 的发病率最高,预后最差。末端脱氧核苷酸转移酶 (TdT) 是一种存在于未成熟淋巴细胞中的核内 DNA 聚合酶 (TdT 阳性),可将 ALL 与成熟淋巴细胞恶性肿瘤区分开来。我们进行了一项多中心回顾性研究,以确定墨西哥裔、白人和美国出生的西班牙裔儿童中 TdT 阴性前 B 细胞急性淋巴细胞白血病 (pre-B ALL) 的发病率,并将 TdT 表达与患者特征和已知的预后因素相关联。对于分类变量,使用 Fisher 确切检验,对于连续变量,使用 Wilcoxon 秩和检验。在具有国家癌症研究所高危疾病的患者中,最常发现 TdT 阴性 pre-B ALL(P=0.014)。TdT 阴性表达也最常与低倍体 pre-B ALL(P=0.001)和 KMT2A 基因重排(P=0.0012)相关。与白人和美国西班牙裔相比,墨西哥儿童 TdT 阴性 ALL 的发病率最高(P<0.001),而且预后不良的特征发生率也更高。这项研究表明,基于种族和民族,TdT 阴性表达、基因组改变和白血病倍性存在显著差异。