臍帯血移植後に多発性脳出血を来したトキソプラズマ関連中枢神経系血管炎  [in Japanese] Toxoplasmosis-associated central nervous system vasculitis accompanied by multiple cerebral hemorrhages developing subsequent to cord blood transplantation  [in Japanese]

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Author(s)

    • 野呂瀬 一美 NOROSE Kazumi
    • 千葉大学大学院医学研究院 感染生体防御学 Department of Infection and Host Defense, Graduate School of Medicine, Chiba University
    • 大野 裕樹 OHNO Yuju
    • 北九州市立医療センター 内科 Department of Internal Medicine, Kitakyushu Municipal Medical Center
    • 今永 博 IMANAGA Hiroshi
    • 北九州市立医療センター 内科 Department of Internal Medicine, Kitakyushu Municipal Medical Center
    • 奥 誠道 OKU Seidou
    • 北九州市立医療センター 内科 Department of Internal Medicine, Kitakyushu Municipal Medical Center
    • 楠元 大岳 KUSUMOTO Hirotake
    • 北九州市立医療センター 内科|南風病院 消化器内科 Department of Internal Medicine, Kitakyushu Municipal Medical Center|Department of Gastroenterology Medicine, Nanpuh Hospital
    • 田宮 貞史 TAMIYA Sadafumi
    • 北九州市立医療センター 病理診断科 Department of Pathological Diagnosis, Kitakyushu Municipal Medical Center
    • 小川 亮介 OGAWA Ryosuke
    • 地域医療機能推進機構九州病院 血液・腫瘍内科 Department of Hematology and Oncology, Japan Community Health Care Organization, Kyushu Hospital
    • 彦坂 健児 HIKOSAKA Kenji
    • 千葉大学大学院医学研究院 感染生体防御学 Department of Infection and Host Defense, Graduate School of Medicine, Chiba University

Abstract

<p>高リスク骨髄異形成症候群の57歳男性に臍帯血移植を施行。Day(D)14に急性graft-versus-host diseaseを発症したためステロイド投与開始し,漸減中のD75にめまいが出現。大脳,小脳,脳幹に多発性脳出血を認め,出血が増大傾向となりD91に脳生検施行。CD3<sup>+</sup>成熟リンパ球の小血管周囲への集簇,リンパ球とマクロファージの血管壁内浸潤を認め中枢神経系血管炎(central nervous system vasculitis, CNSV)と診断。大量ステロイド療法を施行後脳出血の進行は停止したが,D113に嚥下障害が出現し,D128に脳浮腫で死亡。追加検査で脳生検試料中にトキソプラズマDNAおよびその急増虫体が検出されたことより,本症例のCNSV発症の原因としてトキソプラズマの関与が示唆された。稀少疾患のCNSVは急激な経過を辿り,移植後の致死的中枢神経系合併症としての認識が必要であり,トキソプラズマ症を含めその原因解明が予後の改善に重要である。</p>

<p>A 57-year-old man with high-risk myelodysplastic syndrome underwent umbilical cord blood transplantation. He began receiving steroids on day 14 for acute graft-versus-host disease, and experienced dizziness on day 75 during gradual dose reduction. Multiple hemorrhages were observed in the cerebrum, cerebellum, and brainstem. His bleeding increased, and he underwent a brain biopsy on day 91. Subsequently, he was diagnosed with central nervous system vasculitis (CNSV) on the basis of the observed aggregation of mature CD3<sup>+</sup> lymphocytes around small vessels and vascular wall invasion by lymphocytes and macrophages. After receiving high-dose steroid therapy, cerebral hemorrhage stopped; however, dysphasia occurred on day 113 and the patient died of cerebral edema on day 128. <i>Toxoplasma</i> DNA and tachyzoites were detected in the brain biopsy specimen during additional examinations; therefore, we suspected that the toxoplasmosis was related to the onset of CNSV. CNSV is a rare, rapidly progressing disease that may present as a fatal post-transplantation central nervous system complication. Investigating the causes of CNSV, including CNSV associated with toxoplasmosis, is critically important for improving the prognosis of patients with CNSV.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 60(2), 118-123, 2019

    The Japanese Society of Hematology

Codes

  • NII Article ID (NAID)
    130007607569
  • NII NACSIS-CAT ID (NCID)
    AN00252940
  • Text Lang
    JPN
  • ISSN
    0485-1439
  • NDL Article ID
    029553006
  • NDL Call No.
    Z19-295
  • Data Source
    NDL  J-STAGE 
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