title: TCL1A gene involvement in T-cell prolymphocytic leukemia in Japanese patients.
abstract: T-cell prolymphocytic leukemia (T-PLL) is a rare type of peripheral T-cell leukemia. In this study, we examined the clinical and biological characteristics of 11 Japanese patients with T-PLL. Median age was 74 years, with male predominance. Median lymphocyte frequency was 85.3% in blood. Physical characteristics were splenomegaly (36.4%), tiny lymph adenopathy (63.6%), skin lesion (9.1%) and pleural effusion (27.3%). Median survival was 30.1 months, despite treatment with various chemotherapeutic modalities. Although complex chromosomal abnormalities were observed in 5 of 11 cases (45.5%), typical 14q32 and Xq28 abnormalities were not detected. TCL1A mRNA expression was observed in 6 of 11 cases (54.5%) on real-time quantitative PCR. In 5 of these 6 cases, flow cytometric analysis and/or immunohistochemistry confirmed the expression of TCLA1 protein. Split signals for the TCL1 region on fluorescence in situ hybridization confirmed rearrangement in 3 out of 7 cases evaluated. These cases corresponded to cases that were positive for TCL1A expression, suggesting that rearrangement of the TCL1 region induced high expression of TCL1A gene. In summary, a substantial number of T-PLL cases in Japan had abnormal expression of TCL1A, probably due to rearrangement of TCL1 region. Expression and/or rearrangement of TCL1A may, therefore, be a useful marker for diagnosing T-PLL, regardless of chromosomal abnormalities.
authors.affiliation: Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan. ayoko@gunma-u.ac.jp
authors.forename: Akihiko
authors.initials: A
authors.lastname: Yokohama
authors.forename: Akio
authors.initials: A
authors.lastname: Saitoh
authors.forename: Hirotaka
authors.initials: H
authors.lastname: Nakahashi
authors.forename: Takeki
authors.initials: T
authors.lastname: Mitsui
authors.forename: Hiromi
authors.initials: H
authors.lastname: Koiso
authors.forename: Yoshitora
authors.initials: Y
authors.lastname: Kim
authors.forename: Hideki
authors.initials: H
authors.lastname: Uchiumi
authors.forename: Takayuki
authors.initials: T
authors.lastname: Saitoh
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Handa
authors.forename: Takahiro
authors.initials: T
authors.lastname: Jimbo
authors.forename: Kayoko
authors.initials: K
authors.lastname: Murayama
authors.forename: Tohru
authors.initials: T
authors.lastname: Sakura
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Murakami
authors.forename: Masamitsu
authors.initials: M
authors.lastname: Karasawa
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Nojima
authors.forename: Norifumi
authors.initials: N
authors.lastname: Tsukamoto
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005500:Follow-Up Studies; D015973:Gene Expression Regulation, Leukemic; D015321:Gene Rearrangement; D056726:Genetic Association Studies; D006801:Humans; D007564:Japan; D015461:Leukemia, Prolymphocytic, T-Cell / Q000235:genetics* / Q000378:metabolism* / Q000401:mortality / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011518:Proto-Oncogene Proteins / Q000235:genetics* / Q000378:metabolism*; D016019:Survival Analysis
chemical_list: D011518:Proto-Oncogene Proteins; C090851:TCL1A protein, human