The efficacy of alemtuzumab for pure red cell aplasia associated with autoimmune polyendocrine syndrome type 1

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  • SATO Michiaki
    Department of Hematology, NTT Medical Center Tokyo Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo
  • SHINO Masahiro
    Department of Hematology, NTT Medical Center Tokyo
  • YOKOYAMA Kazuaki
    Department of Hematology/Oncology, Research Hospital, The Institute of Medical Science, The University of Tokyo
  • ISHIDA Taiki
    Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo
  • HIRAO Masako
    Department of Hematology, NTT Medical Center Tokyo
  • KAMODA Yoshimasa
    Department of Hematology, NTT Medical Center Tokyo
  • IIZUKA Hiromitsu
    Department of Hematology, NTT Medical Center Tokyo
  • KIDA Michiko
    Department of Hematology, NTT Medical Center Tokyo
  • IMOTO Seiya
    Health Intelligence Center, The Institute of Medical Science, The University of Tokyo
  • TOJO Arinobu
    Department of Hematology/Oncology, Research Hospital, The Institute of Medical Science, The University of Tokyo
  • USUKI Kensuke
    Department of Hematology, NTT Medical Center Tokyo

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Other Title
  • 自己免疫性多発内分泌腺症1型に合併した赤芽球癆に対するalemtuzumabの有効性
  • 自己免疫性多発内分泌腺症1型に合併した赤芽球癆に対するalemtuzumabの有効性 : 第15回日本血液学会関東甲信越地方会 優秀演題
  • ジコ メンエキセイ タハツ ナイブンピツセンショウ 1ガタ ニ ガッペイ シタ アカ ガキュウロウ ニ タイスル alemtuzumab ノ ユウコウセイ : ダイ15カイ ニホン ケツエキ ガッカイ カントウ コウシンエツチホウカイ ユウシュウ エンダイ

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Abstract

<p>We present a case of a 41-year-old woman who was diagnosed with autoimmune polyendocrine syndrome type 1 (APS-1) at the age of 2. She developed severe anemia and was diagnosed with pure red cell aplasia (PRCA) and T-cell large granular lymphocyte leukemia at the age of 34. The pathogenesis of APS-1 is based on the presence of an inactive mutation in the autoimmune regulator gene on thymic medullary epithelial cells. It is thought that the autoimmune T cells generated by impaired negative selection in the thymus induce PRCA. The patient was treated with immunosuppressive therapy (ciclosporin, antithymocyte globulin, prednisolone, and cyclophosphamide) for a long time by her previous doctor. After a long period of remission and exacerbation, she became dependent on blood transfusion approximately at the age of 40 and was transferred to our hospital. At our hospital, alemtuzumab treatment resulted in the disappearance of large granular lymphocytes and improvement of anemia. We report this case as a valuable demonstration of the efficacy of alemtuzumab for treating PRCA associated with APS-1.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 63 (3), 189-193, 2022

    The Japanese Society of Hematology

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