Good’s syndrome developing hemophagocytic lymphohistiocytosis following thymectomy

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  • YAMADA Michiko
    Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital
  • YOSHIDA Masahiro
    Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital
  • OIWA Shutaro
    Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital
  • WATANABE Daichi
    Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital
  • SAGA Junya
    Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital
  • YAMADA Shota
    Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital
  • ONO Michihiro
    Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital
  • ABE Tomoyuki
    Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital
  • FUJII Shigeyuki
    Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital
  • MAEDA Masahiro
    Dept. of Gastroenterology and Hematology/Clinical Oncology, Internal Medicine, Steel Memorial Muroran Hospital
  • FUJITA Miri
    Dept. of Pathology and Clinical Laboratory, Steel Memorial Muroran Hospital
  • IYAMA Satoshi
    Dept. of Hematology, Sapporo Medical University
  • KATO Junji
    Dept. of Medical Oncology, Sapporo Medical University

Bibliographic Information

Other Title
  • 胸腺摘出術後に血球貪食性リンパ組織球症を合併したGood症候群
  • 症例報告 胸腺摘出術後に血球貪食性リンパ組織球症を合併したGood症候群
  • ショウレイ ホウコク キョウセン テキシュツ ジュツゴ ニ ケッキュウドンショクセイ リンパ ソシキキュウショウ オ ガッペイ シタ Good ショウコウグン

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Abstract

<p>This report presents the case of a 68-year-old female patient previously diagnosed with thymoma by her local doctor. She was referred to our hospital for surgery, and the thymoma was removed and diagnosed as a World Health Organization (WHO) classification type AB thymoma. After surgery, she experienced general malaise, a loss of appetite, and weight loss, so she visited our hospital in May 2019. A blood test showed hypogammaglobulinemia and low B lymphocytes. A bone marrow examination revealed no morphological abnormalities. Flow cytometric analysis indicated a marked decrease in both the B cell-related surface markers CD19 and CD20 and the T cell-related surface marker CD4, and the CD4/CD8 ratio was also low. She was diagnosed with Good’s syndrome, and immunoglobulin replacement therapy was administered. She subsequently developed hemophagocytic lymphohistiocytosis (HLH) due to infection and was treated according to the HLH2004 protocol, but she finally succumbed to multiple organ damage as a result of sepsis. Given that Good’s syndrome is associated with both humoral and cellular immune dysfunctions, affected patients tend to develop severe infections and have a poor prognosis. In such cases, early detection, regular immunoglobulin replacement therapy, and infection prevention therapies are important.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 61 (3), 268-273, 2020

    The Japanese Society of Hematology

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