Nomura S, Koshikawa K, Hamamoto K, Okubo S, Yasunaga K
First Department of Internal Medicine, Kansai Medical University.
Rinsho Ketsueki. 1992 Sep;33(9):1242-7.
A 21-year-old woman was admitted to our hospital because of high fever, cough, and headache. Other clinical manifestations included hepatosplenomegaly, anemia, leukopenia, and mild liver dysfunction. As she had been diagnosed to have systemic lupus erythematosus (SLE) previously, we first suspected that this disease had become exacerbated. However, the titers of anti-EB virus (VCA-IgG) and some other viruses were high on admission, and hemophagocytosis by macrophages was observed on bone marrow examination. Therefore, she was diagnosed to have virus-associated hemophagocytic syndrome (VAHS). Prednisolone therapy was then initiated, but the patient responded poorly. We next tried gamma globulin, and the clinical findings and laboratory data were improved. Our patient showed a very favourable clinical course following gamma globulin therapy, suggesting that steroid and gamma globulin should be considered as a treatment even in the early stages of this syndrome in adults.
一名21岁女性因高热、咳嗽和头痛入院。其他临床表现包括肝脾肿大、贫血、白细胞减少和轻度肝功能障碍。由于她之前被诊断患有系统性红斑狼疮(SLE),我们首先怀疑该病病情加重。然而,入院时抗EB病毒(VCA-IgG)及其他一些病毒的滴度较高,骨髓检查发现巨噬细胞噬血现象。因此,她被诊断为病毒相关性噬血细胞综合征(VAHS)。随后开始使用泼尼松龙治疗,但患者反应不佳。我们接着尝试了丙种球蛋白,临床症状和实验室数据得到改善。我们的患者在丙种球蛋白治疗后呈现出非常良好的临床病程,这表明即使在成人该综合征的早期阶段,也应考虑将类固醇和丙种球蛋白作为一种治疗方法。