Successful cord blood transplantation in a patient with adult-onset common variable immunodeficiency

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  • HARUTANI Yuhei
    Department of Hematology/Oncology, Wakayama Medical University
  • TAMURA Shinobu
    Department of Hematology/Oncology, Wakayama Medical University Department of Hematology/Oncology, Kinan Hospital
  • KOBATA Hiroshi
    Department of Hematology/Oncology, Wakayama Medical University
  • OIWA Takehiro
    Department of Hematology/Oncology, Wakayama Medical University
  • YAMASHITA Yusuke
    Department of Hematology/Oncology, Wakayama Medical University
  • MUSHINO Toshiki
    Department of Hematology/Oncology, Wakayama Medical University
  • HOSOI Hiroki
    Department of Hematology/Oncology, Wakayama Medical University
  • MURATA Shogo
    Department of Hematology/Oncology, Wakayama Medical University
  • NISHIKAWA Akinori
    Department of Hematology/Oncology, Wakayama Medical University
  • IMAI Kohsuke
    Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • MORIO Tomohiro
    Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • SONOKI Takashi
    Department of Hematology/Oncology, Wakayama Medical University

Bibliographic Information

Other Title
  • 臍帯血移植が奏効した成人型分類不能型免疫不全症
  • 症例報告 第107回近畿血液学地方会 優秀演題 臍帯血移植が奏効した成人型分類不能型免疫不全症
  • ショウレイ ホウコク ダイ107カイ キンキ ケツエキガク チホウカイ ユウシュウ エンダイ サイタイケツ イショク ガ ソウコウ シタ セイジンガタ ブンルイ フノウガタ メンエキ フゼンショウ

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Description

<p>Common variable immunodeficiency (CVID) is the most frequently diagnosed congenital immunodeficiency and is characterized by dysfunctional antibody production. It often occurs at the age of ≥10 years. Here we reported a case of a 46-year-old man confirmed with adult-onset CVID. He was effectively treated with cord blood transplant (CBT). The patient was observed with repeated upper respiratory infection a few years back and was referred to our department owing to a marked decrease in neutrophil counts and progression of anemia. Laboratory tests confirmed hypogammaglobulinemia, but no autoantibodies were detected. Bone marrow aspiration showed a hypocellular marrow with predominantly mature lymphocytes. T-cell receptor excision circle assay revealed a reduction in T-cell neogenesis. Further, multicolor flow cytometry analysis revealed a low differentiation of B cells; subsequently, CVID was confirmed in the patient. The patient had a severe clinical course and therefore, received CBT for the treatment. After the transplantation, the hematopoiesis was restored and the serum immunoglobulin levels returned to normal. The patient exhibited a favorable clinical course. Nevertheless, there is no precise definition to establish the disease concept of CVID. Also, most of the potential cases are predominantly reported in adults. Therefore, further data on cases with CVID should be accumulated to establish the diagnostic criteria as well as treatment modalities.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 59 (3), 293-299, 2018

    The Japanese Society of Hematology

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