Transformation of severe aplastic anemia to myelodysplastic syndrome with monosomy 7 in a patient who achieved transfusion independence after immunosuppressive therapy

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  • 免疫抑制療法後monosomy 7を伴う骨髄異形性症候群に移行し，輸血非依存性となった重症再生不良性貧血
  • 症例 免疫抑制療法後monosomy 7を伴う骨髄異形成症候群に移行し,輸血非依存性となった重症再生不良性貧血
  • ショウレイ メンエキ ヨクセイ リョウホウ ゴ monosomy 7 オ トモナウ コツズイイケイセイ ショウコウグン ニ イコウ シ ユケツ ヒイゾンセイ ト ナッタ ジュウショウ サイセイ フリョウセイ ヒンケツ

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Abstract

A 72-year-old woman was given a diagnosis of severe aplastic anemia, and treated with anabolic steroid and cyclosporin A starting in October 1996. Because this treatment was ineffective, antithymocyte globulin (ATG) therapy was started in September 1997. In May 1998, chromosome analysis revealed transformation to myelodysplastic syndrome (MDS), refractory anemia with excess of blasts with monosomy 7 in 60% of metaphase cells. The patient showed gradual hematologic improvement and became transfusion independent. Despite progression to acute myeloid leukemia (FAB-M6) with monosomy 7 in 100% of metaphase cells in December 1998, the hemoglobin level recovered to 13.2g/dl. In May 1999 the blasts increased rapidly and transformation to acute myelomonocytic leukemia (FAB-M4) was diagnosed. The patient was treated with low-dose Ara-C and aclarubicin with no improvement and died in August 1999. This case demonstrated the transformation of severe aplastic anemia to acute myeloid leulemia via MDS with monosomy 7 associated with transfusion independence after immunosuppressive therapy. These findings suggested a close relationship between aplastic anemia and hypoplastic MDS and the possibility of hematologic improvement based on the growth advantage of abnormal clones.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 41 (4), 347-353, 2000

    The Japanese Society of Hematology

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