Successful treatment of pure red cell aplasia with cyclosporin in a patient with T-cell large granular lymphocytic leukemia harboring the <i>STAT3</i> D661V mutation

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  • ADACHI Masaaki
    Hematology Division, JCHO Sapporo Hokushin Hospital
  • YOSHIDA Kenichi
    Pathology and Tumor Biology, Graduate School of Medicine, Kyoto University
  • SHIRAISHI Yuichi
    Human Genome Center, Institute of Medical Science, The University of Tokyo
  • CHIBA Kenichi
    Human Genome Center, Institute of Medical Science, The University of Tokyo
  • MIYANO Satoru
    Human Genome Center, Institute of Medical Science, The University of Tokyo
  • OGAWA Seishi
    Pathology and Tumor Biology, Graduate School of Medicine, Kyoto University

Bibliographic Information

Other Title
  • 赤芽球癆に対しシクロスポリンが奏効したSTAT3遺伝子D661V変異を有するT細胞大顆粒リンパ球性白血病
  • 症例報告 赤芽球癆に対しシクロスポリンが奏効したSTAT3遺伝子D661V変異を有するT細胞大顆粒リンパ球性白血病
  • ショウレイ ホウコク アカ ガキュウロウ ニ タイシ シクロスポリン ガ ソウコウ シタ STAT3 イデンシ D661V ヘンイ オ ユウスル Tサイボウ ダイ カリュウ リンパキュウセイ ハッケツビョウ
  • [Successful treatment of pure red cell aplasia with cyclosporin in a patient with T-cell large granular lymphocytic leukemia harboring the STAT3 D661V mutation].

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Description

<p>T-cell large granular lymphocyte (T-LGL) leukemia is a chronic T-cell monoclonal disease that is occasionally associated with pure red cell aplasia (PRCA). A 71-year-old previously healthy man complained of physical fatigue and exhibited anemia (hemoglobin, 10.5 g/dl) with lymphocytosis (76%) showing LGL. The LGL cells expressed CD3, CD7, CD8, and T-cell receptor (TCR) -α/β. TCR-β/γ gene rearrangement was positive. He was thus diagnosed with CD8＋ T-LGL leukemia. Anemia progressed with low reticulocyte count (0.11%), and the patient became blood transfusion-dependent, but no distinct abnormality caused the anemia. Bone marrow aspiration revealed an increase in lymphocytes (33.6%) and a decrease in erythroblasts (M/E ratio, 6.1). He was thus diagnosed with T-LGL-associated PRCA. Oral cyclosporin A administration resulted in prompt improvement of anemia, suggesting its high sensitivity. Whole-exome sequencing of his peripheral blood DNA revealed somatic mutations in 33 genes, including the STAT3 gene, implying their roles in T-LGL leukemia.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 60 (1), 39-45, 2019

    The Japanese Society of Hematology

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