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Immunophenotypic analysis of cerebrospinal fluid reveals concurrent development of ATL in the CNS of a HAM/TSP patient

  • Reina Takeda
  • Tomohiro IshigakiEmail author
  • Nobuhiro Ohno
  • Kazuaki Yokoyama
  • Toyotaka Kawamata
  • Tomofusa Fukuyama
  • Natsumi Araya
  • Yoshihisa Yamano
  • Kaoru Uchimaru
  • Arinobu Tojo
Case Report

Abstract

Both adult T-cell leukemia/lymphoma (ATL) and human T-cell leukemia virus type 1 (HTLV-1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) can be induced by HTLV-1, but concurrent development has been rarely reported. We present the case of a 55-year-old female who developed cranial nerve symptoms after a 20-year history of HAM/TSP. Although multiple white matter lesions were observed on brain magnetic resonance imaging, no abnormalities were seen on a systemic computed tomography scan. Quantitative flow-cytometric analysis of cell populations in the cerebrospinal fluid (CSF) revealed that most of the infiltrating cells were not inflammatory cells, but HTLV-1-infected CD4+ CADM-1+ T-cells completely lacking CD7 expression. As stepwise downregulation of CD7 is correlated with disease progression from HTLV-1 carrier to aggressive ATL, the CSF cells were classified as aggressive ATL; these cells exhibited a more progressed phenotype than those in peripheral blood (PB). HAM/TSP disease activity was estimated to be low. From these and other examinations, we made a diagnosis of acute-type ATL, which unusually developed in the central nervous system at initial onset prior to systemic progression. In ATL cases with a challenging diagnosis, immunophenotypic characterization of CSF and PB is valuable for differential diagnosis and understanding disease status.

Keywords

Adult T-cell leukemia/lymphoma (ATL) HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP) Multi-color flow cytometry Central nervous system involvement 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare no conflict of interest.

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Copyright information

© Japanese Society of Hematology 2020

Authors and Affiliations

  • Reina Takeda
    • 1
  • Tomohiro Ishigaki
    • 1
    • 2
    Email author
  • Nobuhiro Ohno
    • 1
    • 3
  • Kazuaki Yokoyama
    • 1
  • Toyotaka Kawamata
    • 1
    • 3
  • Tomofusa Fukuyama
    • 1
    • 4
  • Natsumi Araya
    • 5
  • Yoshihisa Yamano
    • 5
  • Kaoru Uchimaru
    • 1
    • 6
  • Arinobu Tojo
    • 1
    • 2
    • 3
  1. 1.Department of Hematology/Oncology, Research Hospital, The Institute of Medical ScienceThe University of TokyoTokyoJapan
  2. 2.Department of Laboratory Medicine, Research Hospital, Institute of Medical ScienceThe University of TokyoTokyoJapan
  3. 3.Division of Molecular Therapy, The Institute of Medical ScienceThe University of TokyoTokyoJapan
  4. 4.Division of Cellular Therapy, The Institute of Medical ScienceThe University of TokyoTokyoJapan
  5. 5.Department of Rare Diseases Research, Institute of Medical ScienceSt. Marianna University School of MedicineKawasakiJapan
  6. 6.Laboratory of Tumor Cell Biology, Department of Computational Biology and Medical Science, Graduate School of Frontier SciencesThe University of TokyoTokyoJapan

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