Dummer R, Geertsen R, Ludwig E, Niederer E, Burg G
Department of Dermatology, University of Zurich Medical School, Switzerland.
Leuk Lymphoma. 1998 Feb;28(5-6):515-22. doi: 10.3109/10428199809058359.
Mycosis fungoides and the Sézary Syndrome are characterized by clonal accumulation of well differentiated T-helper memory cells in the skin and, in the case of the Sézary Syndrome, also in the blood and lymph nodes. Well known immunological abnormalities in patients with MF and SS include reduced delayed type hypersensitivity, decreased proliferation upon stimulation with mitogens of the peripheral blood mononuclear cells, elevated IgE or IgA serum levels and eosinophilia. These abnormalities can be explained by the predominance of T-helper 2 cytokines. Clonal T-lymphocytes purified from the peripheral blood of SS patients transcribe mainly IL-10 and IL-5. They can be CD7 positive or negative. These clonal T cells express the accessory factor-1 (AF-1) or Interferon gamma receptor beta-chain that is essential for Interferon gamma signalling. These results imply perspectives for new therapeutical approaches, such as IL-12 or chimeric fusion proteins.
蕈样肉芽肿和塞扎里综合征的特征是皮肤中分化良好的T辅助记忆细胞克隆性积聚，在塞扎里综合征中，血液和淋巴结中也有这种情况。蕈样肉芽肿和塞扎里综合征患者中众所周知的免疫异常包括迟发型超敏反应降低、外周血单核细胞受丝裂原刺激后增殖减少、血清IgE或IgA水平升高以及嗜酸性粒细胞增多。这些异常可以用T辅助2细胞因子占优势来解释。从塞扎里综合征患者外周血中纯化的克隆性T淋巴细胞主要转录IL-10和IL-5。它们可以是CD7阳性或阴性。这些克隆性T细胞表达辅助因子-1（AF-1）或对干扰素γ信号传导至关重要的干扰素γ受体β链。这些结果为新的治疗方法，如IL-12或嵌合融合蛋白，提供了前景。
