Unclassified mature T cell leukemia with cerebriform nuclei

DOI Web Site 15 References
  • TAKIZAWA Makiko
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine
  • MATSUSHIMA Takafumi
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine
  • YOKOHAMA Akihiko
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine
  • HANDA Hiroshi
    Department of Laboratory Sciences, School of Health Sciences, Gunma University
  • TSUKAMOTO Norifumi
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine
  • KARASAWA Masamitsu
    Division of Blood Transfusion, Gunma University Hospital
  • MURAKAMI Hirokazu
    Department of Laboratory Sciences, School of Health Sciences, Gunma University
  • NOJIMA Yoshihisa
    Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine

Bibliographic Information

Other Title
  • 脳回状核を有する分類不能成熟Ｔ細胞性白血病
  • 症例 脳回状核を有する分類不能成熟T細胞性白血病
  • ショウレイ ノウ カイジョウカク オ ユウスル ブンルイ フノウ セイジュク Tサイボウセイ ハッケツビョウ

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Description

A 53 year-old male visited our hospital for evaluation of his leukocytosis, which was first diagnosed more than 6 years previously. He was asymptomatic and there were no remarkable findings on physical and laboratory examinations except for the lymphocytosis. Abnormal lymphocytes with deep folded nuclei were seen on light microscopy, whose phenotype was CD3+, CD4-, CD8-, CD7-, CD16-, CD56-, CD45RO+ and CD45RA-. Electron microscopy revealed ‘cerebriform nuclei’ which were characteristic of Sézary cells. Adult T cell leukemia (ATL) and Sézary syndrome (SS) were ruled out because of the negative HTLV-1 test and the absence of skin lesions, respectively. T-prolymphocytic leukemia (T-PLL), which is characterized by a marked increase in leukocytes having a CD7-phenotype and a progressive fatal course, was also excluded. Recently, the TCL1 onco-protein has been shown to be overexpressed in progressive T-PLL but not in other mature T cell leukemias including Sézary syndrome. Peripheral mononuclear cells in the present patient did not overexpress TCL1. In its morphology and phenotypes, our case resembled ‘Sézary cell leukemia (SCL)’ but the clinical course was much more indolent. This case did not match any of the mature T cell leukemias defined in the WHO classification.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 46 (7), 486-491, 2005

    The Japanese Society of Hematology

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