Hypereosinophilic syndromeの診断から12年後に発症したCD19陽性acute myeloblastic leukemia  [in Japanese] CD19-positive acute myeloblastic leukemia developed 12 years after the onset of hypereosinophilic syndrome  [in Japanese]

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症例は46歳,男性。34歳時に好酸球増加(WBC 38,200/μ<i>l</i>, 好酸球74%)と心嚢水貯留を認め,hypereosinophilic syndrome (HES)と診断された(染色体は正常核型)。化学療法にて軽快し以後無治療であったが,発症から12年後,発熱のため当院入院。WBC 3,000/μ<i>l</i>(芽球70%, 好酸球3%),骨髄は95%が芽球であった。芽球はmyeloperoxydase (MPO)染色陰性,CD13, CD19, CD34, HLA-DR, cytoplasmic MPOが陽性でありCD19陽性acute myeloblastic leukemiaと診断した。核型は46, XY, t(6;21)(q13;q22), add(7)(q11)で,AML1遺伝子の再構成は認めなかった。AdVP療法にて完全寛解となる。21q22と7qの異常を認めたことから,HESに対する化学療法が白血病の発症に関与した可能性も考えられた。

We report a rare case of hypereosinophilic syndrome (HES) that developed to acute myeloblastic leukemia (AML). The patient, a 34-year-old man, presented with eosinophilia of unknown origin (white blood cells 38,200/μ<i>l</i> with 74% eosinophils) and pericardial effusion, and was diagnosed as having HES with a normal karyotype. He received four cycles of combination chemotherapy including cyclophosphamide, cytosine arabinoside and vindesine, and thereafter remained in remission. After 12 years, he was referred to our hospital because of fever and malaise. On admission, CBC showed white blood cells 3,000/μ<i>l</i> with 70% myeloblasts and 3% eosinophils. The bone marrow was hypercellular with 95% blasts, which were negative for myeloperoxidase (MPO) staining. Immunophenotype analysis revealed that the cells were positive for CD13, CD19, CD34, HLA-DR and cytoplasmic MPO. CD19-positive AML was diagnosed. Cytogenetic analysis showed 46, XY, t(6;21)(q13;q22), add(7)(q11) in 19 of 20 metaphase spreads. Rearrangement of the AML1 gene at 21q22 and fusion of the <i>BCR/ABL</i> gene could not be detected by fluorescence in situ hybridization analysis. The patient received combination chemotherapy and achieved a complete remission. Chromosome aberrations involving 7q as well as 21q22 suggested that the initial chemotherapy for HES might have been implicated in the pathogenesis of acute leukemia in this case.


  • Rinsho Ketsueki

    Rinsho Ketsueki 41(9), 723-728, 2000-09-30

    The Japanese Society of Hematology

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Cited by:  1


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