Division of Hematology and Oncology, Department of Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, 830-0011, Japan.
Department of Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan.
Int J Hematol. 2021 Jun;113(6):815-822. doi: 10.1007/s12185-021-03116-8. Epub 2021 Mar 16.
Acute lymphoblastic leukemia (ALL) is a common neoplasm in children, but less frequent in adults. Since information on clinical features and genetics of adult ALL in Japan is limited, we analyzed 215 subjects aged 16-65 years with untreated ALL enrolled in the Fukuoka Blood & Marrow Transplant Group studies ALL MRD 2002 and 2008. The prevalence of ALL was bimodal, with the larger group aged 56-65 years. Immunophenotypic characterization showed B-lineage is more frequent than T-lineage ALL (78.6 vs 13.0%), with age-related differences. The proportion with BCR-ABL1 rearrangement increased progressively with age, up to 55.7% among subjects aged over 56-65 years. Rearrangements involving the KMT2A gene, ETV6-RUNX1, and TCF3-PBX1 were rare in this study cohort. The overall incidence of hyperdiploidy was only 1.7%, and there were no cases with hypodiploidy. Overall survival varied by age and cytogenetics. Older subjects and those with BCR-ABL1 tended to have inferior outcomes. In this epidemiological study of Japanese adult ALL, the majority of subjects had B-lineage ALL, the T-cell phenotype was most frequent in those aged 16-25, and BCR-ABL1 rearrangement was very common, with prevalence increasing with age. These types of adult ALL are potentially manageable with targeted therapies.
急性淋巴细胞白血病（ALL）是儿童常见的恶性肿瘤，但在成人中较少见。由于日本成人 ALL 的临床特征和遗传学信息有限，我们分析了未接受治疗的 215 名年龄在 16-65 岁的 ALL 患者，这些患者均入组了福冈血液与骨髓移植组的 ALL MRD 2002 和 2008 研究。ALL 的发病呈双峰分布，较大的一组为 56-65 岁。免疫表型特征表明 B 细胞系比 T 细胞系 ALL 更为常见（78.6%比 13.0%），且存在与年龄相关的差异。具有 BCR-ABL1 重排的比例随年龄逐渐增加，在年龄超过 56-65 岁的患者中高达 55.7%。在本研究队列中，KMT2A 基因、ETV6-RUNX1 和 TCF3-PBX1 涉及的重排很少见。总体高倍体发生率仅为 1.7%，且无低倍体病例。总生存因年龄和细胞遗传学而异。年龄较大的患者和具有 BCR-ABL1 的患者往往预后较差。在这项日本成人 ALL 的流行病学研究中，大多数患者为 B 细胞系 ALL，T 细胞表型在 16-25 岁年龄组最为常见，BCR-ABL1 重排非常常见，且随着年龄的增加而增加。这些类型的成人 ALL 可能可以通过靶向治疗来控制。
