Tesch H, Bohlen H, Wolf J, Engert A
Klinik I für Innere Medizin, Universität Köln.
Med Klin (Munich). 1998 Feb 15;93(2):82-90. doi: 10.1007/BF03043282.
Although the pathogenesis of Hodgkin's disease is not clear, molecular analyses reveal characteristic features. EBV infection can be demonstrated in more than 50% of cases at the DNA or protein level. Recently, immunoglobulin gene rearrangements were found in single Hodgkin and Reed-Sternberg cells. Sequence analyses revealed that the rearranged Ig genes have frequently somatic mutations, which indicate that the cells are derived from the germinal center. These rearrangements may be used as defined markers to detect residual disease after chemotherapy. Modern polychemotherapy regimen and radiotherapy are very effective, and 60-90% of patients, depending on stage of the disease and risk factors, can be cured. Salvage therapy for relapsed patients including high-dose chemotherapy with autologous stem cell support frequently results in remission although duration is frequently short. New immunotherapy strategies with immunotoxins or bispecific antibodies are currently analysed in clinical studies.
尽管霍奇金淋巴瘤的发病机制尚不清楚，但分子分析揭示了其特征性特点。超过50%的病例在DNA或蛋白质水平可检测到EB病毒感染。最近，在单个霍奇金和里德-斯腾伯格细胞中发现了免疫球蛋白基因重排。序列分析显示，重排的Ig基因常有体细胞突变，这表明这些细胞来源于生发中心。这些重排可作为明确的标志物用于检测化疗后的残留疾病。现代联合化疗方案和放疗非常有效，根据疾病分期和危险因素，60%至90%的患者可被治愈。复发患者的挽救治疗，包括采用自体干细胞支持的大剂量化疗，虽缓解期通常较短，但常可导致缓解。目前临床研究正在分析采用免疫毒素或双特异性抗体的新免疫治疗策略。
