Immune microenvironment and prognostic factors in pediatric peripheral T-cell lymphomas

2020 Fiscal Year Final Research Report

• Project/Area Number: 18K07006
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 49020:Human pathology-related
• Research Institution: Saitama Children's Medical Center
• Principal Investigator: Nakazawa Atsuko 埼玉県立小児医療センター (臨床研究部), 臨床研究部, 部長 (90227736)
• Co-Investigator(Kenkyū-buntansha): 大島 孝一 久留米大学, 医学部, 教授 (50203766)
• Project Period (FY): 2018-04-01 – 2021-03-31
• Keywords: 小児リンパ腫 / 末梢性T細胞リンパ腫 / 遺伝子変異

Outline of Final Research Achievements

Nine cases of 1875 pediatric lymphoma cases were EBER-negative peripheral T-cell lymphomas (PTCL). Six cases were CD4-negative and CD8-positive, one case was double positive, and two cases were double negative. Seven cases were positive for cytotoxic molecules. All cases tested were included in a CCR3 pattern, which showed better prognosis compared to CXCR3 or CCR4 pattern. TNS1, ZFHX3,TET2, and NCOA2 mutation were detected, but no case had p53 or VAV1 gene alteration. JAK-STAT pathway related gene abnormality was not observed. It is suggested that pediatric EBER-negative PTCLs have much better prognosis than adult PTCL due to different genetic background.

Free Research Field

小児リンパ腫

Academic Significance and Societal Importance of the Research Achievements

非常にまれな小児のEBER陰性末梢性T細胞リンパ腫(PTCL)についての研究で、成人とは異なる遺伝子背景、免疫微小環境を有することが示唆された。予後予測や治療の選択に関係するデータが得られた。