Hepatitis-associated aplastic anemia preceded by a hemophagocytic syndrome-like state

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Other Title
  • 血球貪食症候群様の病態を経て発症した肝炎後再生不良性貧血
  • 症例 血球貪食症候群様の病態を経て発症した肝炎後再生不良性貧血
  • ショウレイ ケッキュウドンショク ショウコウグン ヨウ ノ ビョウタイ オ ヘテ ハッショウ シタ カンエン ゴ サイセイ フリョウセイ ヒンケツ

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Description

A 21-year-old man was admitted to our hospital for acute hepatitis of unknown cause. His liver function improved with rest, but worsened 2 months later. He developed a high fever and pancytopenia. The serum level of cytokines including TNF-α, IFN-γ, IL-6, and M-CSF was elevated, and hemophagocytes were seen in bone marrow. These findings suggested a hemophagocytic syndrome-like state. With prednisolone, γ-globulin, and G-CSF, the high fever disappeared and the patient's liver function gradually recovered. However, the severe pancytopenia persisted. The bone marrow became acellular with a small number of hemophagocytes, and hepatitis-associated aplastic anemia was diagnosed. After immunosuppressive therapy with ATG, CyA and G-CSF was started, and the patient showed hematopoietic reconstitution. The bone marrow CD4+/CD8+ lymphocyte ratio recovered to within the normal range, and the serum cytokines including TNF-α and IFN-γ decreased. The increase in serum cytokines, particularly TNF-α and INF-γ, as well as the presence of activated T cells associated with the preceding hemophagocytic syndrome-like state may have predisposed this patient to aplastic anemia.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 44 (10), 1010-1014, 2003

    The Japanese Society of Hematology

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