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结节性淋巴细胞为主型霍奇金淋巴瘤(NLPHL)原发于单克隆性B细胞,免疫组织化学特征包括淋巴肿瘤细胞(BCL6、CD20)及微环境细胞(CD23、IgD、PD1)的标志。NLPHL转化为侵袭性淋巴瘤前后的肿瘤细胞可能源于共同的前体细胞,10年累计转化率为7%～12%,并随着时间的增加而增加。目前ⅠA或ⅡA期NLPHL治疗标准方案为受累野放疗,但ABVD方案治疗在临床研究中已初步显示出优势,认为利妥西单抗应用于初治和复发NLPHL的患者均可得到较好疗效。然而转化淋巴瘤对治疗反应性较未转化者差,尚无标准治疗方案。
Nodular lymphocyte predominant Hodgkin’s lymphoma (NLPHL) is primary in monoclonal B cells. Immunohistochemical features include lymphocytic (BCL6, CD20) and microenvironmental cells (CD23, IgD, PD1) Sign. Tumor cells before and after transformation of NLPHL into invasive lymphoma may originate from common precursor cells with a 10-year cumulative conversion of 7% to 12% and an increase over time. At present, the standard protocol of NLPHL for stage ⅠA or ⅡA is involved field radiotherapy. However, ABVD regimen has shown preliminary advantages in clinical studies. It is believed that the efficacy of rituximab in patients with both untreated and relapsed NLPHL is better. However, the conversion of lymphoma response to treatment was worse than those who did not, there is no standard treatment.