Clinical features and chromosomal/genetic aberration in adult acute lymphoblastic leukemia in Japan: results of Fukuoka Blood & Marrow Transplant Group Studies ALL MRD 2002 and 2008

• Published in: International journal of hematology Vol. 113; no. 6; pp. 815 - 822
• Main Authors: Morishige, Satoshi, Miyamoto, Toshihiro, Eto, Tetsuya, Uchida, Naoyuki, Kamimura, Tomohiko, Miyazaki, Yasuhiko, Ogawa, Ryosuke, Okumura, Hirokazu, Fujisak, Tomoaki, Iwasaki, Hiromi, Kawano, Noriaki, Wake, Atsushi, Ohta, Takanori, Takamatsu, Yasushi, Kurokawa, Toshiro, Ito, Yoshikiyo, Maeda, Takahiro, Akashi, Koichi, Nagafuji, Koji
• Format: Journal Article
• Language: English
• Published: Singapore Springer Singapore 01-06-2021; Springer Nature B.V
• Subjects: Acute lymphoblastic leukemia; Age; Blood; Cytogenetics; Epidemiology; Genetics; Hematology; Hypodiploidy; Leukemia; Lymphatic leukemia; Lymphocytes T; Medicine; Medicine & Public Health; MLL protein; Oncology; Original Article; Phenotypes; Runx1 protein; Japan; Cytogenetics; Adult; Clinical features; Acute lymphoblastic leukemia; BCR-ABL1
• ISSN: 0925-5710; 1865-3774
• DOI: 10.1007/s12185-021-03116-8

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.