Refractory thrombocytopeniaで発症し,多彩な炎症像を呈した慢性骨髄単球性白血病移行例  [in Japanese] Progression of refractory thrombocytopenia to chronic myelomonocytic leukemia accompanied by various inflammatory reactions  [in Japanese]

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Author(s)

    • 五明 広志 GOMYO Hiroshi
    • 兵庫県立成人病センター血液内科 Hematology/Oncology Division, Department of Medicine, Hyogo Medical Center for Adults
    • 村山 徹 MURAYAMA Tohru
    • 兵庫県立成人病センター血液内科 Hematology/Oncology Division, Department of Medicine, Hyogo Medical Center for Adults
    • 水野 石一 MIZUNO Ishikazu
    • 兵庫県立成人病センター血液内科 Hematology/Oncology Division, Department of Medicine, Hyogo Medical Center for Adults
    • 小泉 民雄 KOIZUMI Tamio
    • 兵庫県立成人病センター血液内科 Hematology/Oncology Division, Department of Medicine, Hyogo Medical Center for Adults
    • 井本 しおん IMOTO Shion
    • 兵庫県立成人病センター血液内科 Hematology/Oncology Division, Department of Medicine, Hyogo Medical Center for Adults

Abstract

症例は51歳,男性。1997年5月,血小板減少(0.4×10<sup>4</sup>/μ<i>l</i>), 出血傾向にて入院となった。骨髄像はやや低形成で巨核球は減少し,micromegakaryocyte, 低分葉巨核球等の形態異常を認め,MDS, RA (refractory thrombocytopenia; RTC)と診断した。染色体分析では,分析された20細胞全てに46, XY, t(5;7)(q31;q22)を認めた。血小板輸血等で経過観察していたが,1998年11月頃より末梢血中白血球,単球数が増加し,同年12月には単球数は3,000/μ<i>l</i>を越え,慢性骨髄単球性白血病(CMML)へ移行した。白血球数の増加とほぼ平行して顔面の有痛性紅斑,眼内炎等,頭頚部領域にさまざまな炎症像を呈した。また,治療目的でIFNαを投与したところ,逆に白血球数,単球数は著増した。5q31を含む染色体異常が,IFNα投与後の白血球,単球数の増加と関係している可能性が疑われた。RTCからCMMLへの移行例は稀であり,本症例の病態と遺伝子異常との関連を解析することが重要と思われた。

A 51-year-old man was admitted for treatment of severe thrombocytopenia in May 1997. A diagnosis of MDS RA (refractory thrombocytopenia; RTC) was made by bone marrow examination, which revealed mild marrow hypoplasia and a reduced number of megakaryocytes accompanied by micromegakaryocytes and hypolobular megakaryocytes. Chromosome analysis demonstrated 46, XY, t(5;7)(q31;q22) in all 20 cells examined. The patient received only supportive therapy including platelet transfusion, until leukocytosis and monocytosis gradually developed in November 1998. In view of a marked increase in the number of monocytes (more than 3,000/μ<i>l</i>), a diagnosis of CMML was made in December 1998. As the leukocytosis progressed, various inflammatory symptoms such as facial erythema and endophthalmitis developed. Administration of interferon alpha (IFNα) unexpectedly worsened the leukocytosis and monocytosis, suggesting abnormal responses of these cells to IFNα. Detailed molecular analysis of these cells might reveal a novel mechanism of leukemogenesis associated with 5q31.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 41(8), 664-670, 2000-08-30

    The Japanese Society of Hematology

References:  16

Codes

  • NII Article ID (NAID)
    10008038103
  • NII NACSIS-CAT ID (NCID)
    AN00252940
  • Text Lang
    JPN
  • Article Type
    NOT
  • ISSN
    04851439
  • NDL Article ID
    6295782
  • NDL Source Classification
    ZS21(科学技術--医学--内科学)
  • NDL Call No.
    Z19-295
  • Data Source
    CJP  NDL  J-STAGE 
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