<i>JAK2</i>-V617F変異を有する二次性骨髄線維症に発症した<i>BCR-ABL1</i>陽性慢性骨髄性白血病  [in Japanese] <i>BCR-ABL1</i>-positive chronic myeloid leukemia emerging in a patient with secondary myelofibrosis harboring the <i>JAK2</i>-V617F mutation  [in Japanese]

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Author(s)

Abstract

<p>53歳の女性。26歳頃より本態性血小板血症として近医にて経過観察されていた。白血球増加,貧血進行により紹介受診し,<i>BCR-ABL1</i>陽性慢性骨髄性白血病と診断された。同時に<i>JAK2</i>-V617F変異も認め,巨大脾腫を伴った。Dasatinibにて至適奏効となるも,巨大脾腫は残存し,骨髄生検では骨髄線維症の所見であった。脾腫の増大に伴い4年5ヶ月目で,IS(国際指標で補正された値)にて分子遺伝学的奏効(MR)<sup>4.5</sup>を確認後,dasatinibを中止,ruxolitinibを開始した。その5ヶ月後の時点で脾腫は顕著に縮小した。本例では,<i>JAK2</i>-V617F変異を有する腫瘍細胞のサブクローンに<i>BCR-ABL1</i>を生じたと推測された。</p>

<p>A 53-year-old woman with a 27-year history of myeloproliferative neoplasms came to our hospital because of a marked white blood cell count increase and progressive anemia. Clinical examination demonstrated positivity for <i>BCR-ABL1</i> and <i>JAK2</i>-V617F mutations. She was given a diagnosis of chronic myeloid leukemia. Using the international scale, a molecular response (MR) <sup>4.5</sup> was achieved after treatment with dasatinib, despite the persistence of marked splenomegaly. The pathological findings of myelofibrosis were demonstrated by bone marrow biopsy. After stopping dasatinib administration for 4 years and 5 months, treatment with ruxolitinib was started. Five months later, the size of her spleen was reduced. We speculated that translocation of <i>BCR-ABL1</i> might have occurred in a sub-clone of the <i>JAK2</i>-V617F mutated tumor clone.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 58(4), 298-302, 2017

    The Japanese Society of Hematology

Codes

  • NII Article ID (NAID)
    130006882220
  • NII NACSIS-CAT ID (NCID)
    AN00252940
  • Text Lang
    JPN
  • ISSN
    0485-1439
  • NDL Article ID
    028193876
  • NDL Call No.
    Z19-295
  • Data Source
    NDL  J-STAGE 
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