Durable remission of T-cell prolymphocytic leukemia with <i>CLEC16A::IL2</i> after allogeneic hematopoietic stem cell transplantation

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  • MOMOSE Haruka
    Department of Hematology, University of Tsukuba Hospital
  • KURITA Naoki
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Institute of Medicine, University of Tsukuba
  • NISHIKII Hidekazu
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Institute of Medicine, University of Tsukuba
  • YUSA Nozomi
    Department of Applied Genomics, Research Hospital, The Institute of Medical Science, The University of Tokyo
  • YOKOYAMA Kazuaki
    Department of Hematology & Oncology, The Institute of Medical Science, The University of Tokyo
  • SHIMIZU Eigo
    Division of Health Medical Intelligence, Human Genome Center, The Institute of Medical Science, The University of Tokyo
  • IMOTO Seiya
    Division of Health Medical Intelligence, Human Genome Center, The Institute of Medical Science, The University of Tokyo
  • NANMOKU Toru
    Department of Laboratory Medicine, University of Tsukuba Hospital
  • MARUYAMA Yumiko
    Department of Hematology, University of Tsukuba Hospital
  • SAKAMOTO Tatsuhiro
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Institute of Medicine, University of Tsukuba
  • YOKOYAMA Yasuhisa
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Institute of Medicine, University of Tsukuba
  • KATO Takayasu
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Institute of Medicine, University of Tsukuba
  • MATSUOKA Ryota
    Department of Diagnostic Pathology, Institute of Medicine, University of Tsukuba
  • OBARA Naoshi
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Institute of Medicine, University of Tsukuba
  • SAKATA-YANAGIMOTO Mamiko
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Institute of Medicine, University of Tsukuba
  • CHIBA Shigeru
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Institute of Medicine, University of Tsukuba

Bibliographic Information

Other Title
  • 同種造血幹細胞移植により長期寛解を得た<i>CLEC16A::IL2</i>融合遺伝子を有するT細胞前リンパ球性白血病
  • 同種造血幹細胞移植により長期寛解を得たCLEC16A::IL2融合遺伝子を有するT細胞前リンパ球性白血病
  • ドウシュ ゾウケツ カンサイボウ イショク ニ ヨリ チョウキカンカイ オ エタ CLEC16A::IL2 ユウゴウ イデンシ オ ユウスル Tサイボウ ゼン リンパキュウセイ ハッケツビョウ

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Abstract

<p>A 64-year-old woman presented with fine motor impairment in both hands. MRI revealed a contrast-enhanced lesion in the medulla oblongata. Lymphoid cells with abnormal blebs were observed and a CD4+/CD8+ double positive (DP) T cell population was detected by flow cytometry (FCM) in the bone marrow (BM) and the peripheral blood (PB). CLEC16A::IL2 fusion gene was identified by whole exome sequencing with DNA prepared from DP T cells. Clonal rearrangement of the T-cell receptor gene and expression of TCL1A protein were detected. This led to a diagnosis of T-cell prolymphocytic leukemia (T-PLL) with central nervous system (CNS) infiltration. Abnormal cells in BM and PB became undetectable on microscopy and FCM, and the CNS lesion disappeared on MRI after second-line therapy with alemtuzumab. Meanwhile, the CLEC16A::IL2 fusion mRNA remained detectable in PB. Allogeneic hematopoietic stem-cell transplantation was performed, and the fusion mRNA has now been undetectable for more than 5 years since transplantation. This is the first report of a T-PLL case with a CLEC16A::IL2 fusion gene.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 65 (1), 35-40, 2024

    The Japanese Society of Hematology

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