Bone marrow findings of childhood aplastic anemia: analyses of 140 cases by central reviewers

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  • HAMA Asahito
    Department of Pediatrics, Nagoya University Graduate School of Medicine
  • YOSHIMI Ayami
    Department of Hematology and Oncology, Freiburg University Children's Hospital
  • SAKAGUCHI Hirotoshi
    Department of Pediatrics, Nagoya University Graduate School of Medicine
  • DOISAKI Sayoko
    Department of Pediatrics, Nagoya University Graduate School of Medicine
  • MURAMATSU Hideki
    Department of Pediatrics, Nagoya University Graduate School of Medicine
  • SHIMADA Akira
    Department of Pediatrics, Nagoya University Graduate School of Medicine
  • TAKAHASHI Yoshiyuki
    Department of Pediatrics, Nagoya University Graduate School of Medicine
  • NOZAWA Kazue
    Department of Transfusion Medicine, St. Luke's International Hospital
  • ITO Masafumi
    Department of Pathology, Japanese Red Cross Nagoya 1st Hospital
  • TSUCHIDA Masahiro
    Division of Pediatric Hematology and Oncology, Ibaraki Children's Hospital
  • MANABE Atsushi
    Department of Pediatrics, St. Luke's International Hospital
  • OHARA Akira
    Department of Transfusion Medicine, Toho University School of Medicine Omori Medical Center Hospital
  • KOJIMA Seiji
    Department of Pediatrics, Nagoya University Graduate School of Medicine

Bibliographic Information

Other Title
  • 小児再生不良性貧血の骨髄像：140例のセントラルレビューによる検討
  • 臨床研究 第71回日本血液学会学術集会 学会奨励賞受賞論文 小児再生不良性貧血の骨髄像--140例のセントラルレビューによる検討
  • リンショウ ケンキュウ ダイ71カイ ニホン ケツエキ ガッカイ ガクジュツ シュウカイ ガッカイ ショウレイショウ ジュショウ ロンブン ショウニ サイセイ フリョウセイ ヒンケツ ノ コツズイゾウ 140レイ ノ セントラルレビュー ニ ヨル ケントウ

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Abstract

The revised WHO classification proposed the term “refractory cytopenia of childhood (RCC)” for children with myelodysplastic syndrome (MDS) with a low blast count. The differential diagnosis between RCC and aplastic anemia (AA) is challenging, especially when bone marrow is hypoplastic and there is no detectable chromosomal abnormality. To reveal the difference between AA and RCC with respect to the clinical and biological features, we retrospectively reviewed the bone marrow smears of 140 patients registered for childhood AA-97 study, which were classified into three groups as follows; the AA group was defined as having no morphologically dysplastic changes; the AA-RCC borderline group was defined as having <10% dysplastic changes in the erythroid lineage only; and the RCC group was defined as having dysplastic changes in more than two cell lineages or >10% in a single cell lineage. The patients were classified into the AA group (n=96, 69%), AA-RCC borderline group (n=20, 14%) and RCC group (n=24, 17%). Most of the patients in the AA group were classified as having very severe disease, whereas most of the patients in the RCC group were classified as non-severe disease. Only 2 patients in the AA group developed acute myeloid leukemia. The response rate to immunosuppressive therapy did not differ among the 3 groups. To demonstrate whether the two diseases are truly different entities, it is necessary to compare molecular backgrounds between the AA and RCC groups.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 52 (8), 653-658, 2011

    The Japanese Society of Hematology

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