Matsunawa Manabu, Kawakami Keiichiro, Hisatake Jun-ichi, Suzuki Junko, Nakamaki Tsuyoshi, Hino Ken-ichiro, Tomoyasu Shigeru
Department of Hematology, Showa University School of Medicine.
Rinsho Ketsueki. 2003 Oct;44(10):1010-4.
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-alpha, IFN-gamma, IL-6, and M-CSF was elevated, and hemophagocytes were seen in bone marrow. These findings suggested a hemophagocytic syndrome-like state. With prednisolone, gamma-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-alpha and IFN-gamma decreased. The increase in serum cytokines, particularly TNF-alpha and INF-gamma, 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.
一名21岁男性因不明原因的急性肝炎入住我院。经休息，其肝功能有所改善，但2个月后病情恶化。他出现高热和全血细胞减少。包括肿瘤坏死因子-α（TNF-α）、干扰素-γ（IFN-γ）、白细胞介素-6（IL-6）和巨噬细胞集落刺激因子（M-CSF）在内的细胞因子血清水平升高，骨髓中可见噬血细胞。这些发现提示存在噬血细胞综合征样状态。使用泼尼松龙、γ-球蛋白和粒细胞集落刺激因子（G-CSF）后，高热消退，患者肝功能逐渐恢复。然而，严重的全血细胞减少持续存在。骨髓变为无细胞状态，有少量噬血细胞，诊断为肝炎相关性再生障碍性贫血。开始使用抗胸腺细胞球蛋白（ATG）、环孢素A（CyA）和G-CSF进行免疫抑制治疗后，患者出现造血重建。骨髓CD4+/CD8+淋巴细胞比值恢复到正常范围内，包括TNF-α和IFN-γ在内的血清细胞因子水平下降。血清细胞因子尤其是TNF-α和干扰素-γ的升高，以及与先前噬血细胞综合征样状态相关的活化T细胞的存在，可能使该患者易患再生障碍性贫血。
