The new approach for Neurofibromatosis type 1 treatment using TNF-alpha antibody

1型神经纤维瘤病（NF-1）患者存在多发皮肤神经纤维瘤，部分神经纤维瘤可巨大增大，发展为周围神经鞘恶性肿瘤，影响患者的生活质量，生命预后差。研究者证实肿瘤坏死因子α (tnf - α)增强培养的人神经纤维瘤细胞的有丝分裂活性。本研究探讨了体内神经纤维瘤对tnf - α的生长反应。给予tnf - α的NF-1模型小鼠的肿瘤生长强于未给予tnf - α的NF-1模型小鼠。特异性tnf - α抗体抑制tnf - α作用导致NF-1模型小鼠神经纤维瘤生长受到抑制的趋势。

The patients with neurofibromatosis type 1 (NF-1) have multiple cutaneous neurofibroma and some neurofibroma may increase in huge size and develop malignant peripheral nerve sheath tumor, which harm patients' quality of life and lead to the dismal vital prognosis. The investigator confirmed that the tumor necrosis factor alpha (TNF-alpha) augments mitogenic activity of cultured human neurofibroma cells. In this study, the growth responses of in vivo neurofibroma to TNF-alpha was invastigated. the tumor growth in the group of NF-1 modelmouse who was given TNF-alpha was stronger than that in the group NF-1 mouse without TNF-alpha administration. Inhibition of the TNF-alpha action with specific TNF-alpha antibody led to the tendency of suppression of neurofibroma growth in NF-1 modelmouse.