Shirono K, Tsuda H
Division of Clinical Haematology and Immunology, Kumamoto City Hospital, Japan.
Eur J Haematol. 1995 Oct;55(4):240-4. doi: 10.1111/j.1600-0609.1995.tb00264.x.
The clinical characteristics of 15 adult patients with virus-associated haemophagocytic syndrome (VAHS) were studied. The patients were 3 males and 12 females with a mean age of 39.5 years (range 20 to 67 years). Seven patients (mean age 48.6 years) were immunosuppressed by drugs or as a result of having malignant or autoimmune disease. Eight patients (mean age 31.6 years) had no underlying diseases. The prognosis of the patients with immunosuppression was poor, as previously reported, and 3 of them died. In younger adult patients with this syndrome who had no underlying immunosuppressive diseases, the prognosis was good even without therapy. The sera from the patients in both groups contained extremely high levels of macrophage colony-stimulating factor (M-CSF) and slightly elevated levels of tumour necrosis factor-alpha (TNF-alpha). These findings suggested that VAHS, can occur in presumably healthy adults and that VAHS can be a more benign condition than previously believed. M-CSF and TNF-alpha may play an important role in the development of the syndrome in both groups.
对15例成人病毒相关性噬血细胞综合征(VAHS)患者的临床特征进行了研究。患者中男性3例,女性12例,平均年龄39.5岁(范围20至67岁)。7例患者(平均年龄48.6岁)因药物或患有恶性或自身免疫性疾病而免疫抑制。8例患者(平均年龄31.6岁)无基础疾病。如先前报道,免疫抑制患者的预后较差,其中3例死亡。在无基础免疫抑制疾病的年轻成年该综合征患者中,即使未经治疗,预后也良好。两组患者的血清中巨噬细胞集落刺激因子(M-CSF)水平极高,肿瘤坏死因子-α(TNF-α)水平略有升高。这些发现提示,VAHS可发生于看似健康的成年人,且VAHS可能比之前认为的更为良性。M-CSF和TNF-α可能在两组综合征的发生发展中起重要作用。