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目的：探讨间变性非霍奇金淋巴瘤(ALCL)的诊断、临床病理特点、治疗方法及预后。方法：复习1994年6月至1998年12月我院原诊断为大细胞淋巴瘤、霍奇金病等病例的HE染色切片及部分免疫组化切片，根据1992版的最新Kiel分类有关ALCL的诊断标准，筛选组织形态符合ALCL，有详细的临床、治疗及随访资料，经单克隆抗体BerH2标记阳性可确诊为ALCL的病例20例，进一步检测LCA、L26、UCHL1，对部分BerH2、L26阳性病例进行CD15的检测。本组患者均采用以CHOP为主的联合化疗方案，无效或4个疗程不能取得完全缓解用解救方案(IMVP16、DHAP)或局部放疗。结果：本组年轻男性发病多见，主要体征为全身浅表淋巴结肿大(80％)，部分可累及纵隔(30％)、腹腔(25％)、盆腔淋巴结(20％)，结外组织也可受累，其中最常见是皮肤溃疡(30％)。确诊时较多患者AnnArbor分期为Ⅲ期(35％)和Ⅳ期(35％)，且有B症状(55％)和LDH水平升高(70％)。全组BerH2阳性，免疫表型以T细胞为主(60％)，部分为B细胞(35％)或非B非T(5％)。近期疗效：有效率(CR＋PR)17例(85％)，其中完全缓解(CR)12例(60％)。1年、2年、3年生存率均为54.9％。首次化疗是否CR、B症状、有巨大病变均为影响生存的因素。结论：ALCL是一种具有特殊免疫表型和临床特点的非霍奇金淋巴?
Objective: To investigate the diagnosis, clinical pathology, treatment and prognosis of anaplastic non-Hodgkin’s lymphoma (ALCL). METHODS: We reviewed HE stained and partially immunohistochemical sections originally diagnosed as large cell lymphoma and Hodgkin’s disease from June 1994 to December 1998 in our hospital. According to the 1992 Kiel classification, the diagnosis of ALCL was performed. Standards, screened histopathology in line with ALCL, detailed clinical, treatment, and follow-up data, 20 patients with ALCL positive by monoclonal antibody BerH2 positive, further testing for LCA, L26, UCHL1, and partial BerH2, L26 positive cases Detection of CD15. In this group of patients, CHOP-based combination chemotherapy regimens were used. Ineffective or 4 courses could not achieve complete remission rescue programs (IMVP16, DHAP) or local radiotherapy. Results: This group of young males was more common. The main signs were superficial lymphadenopathy (80%), partial mediastinum (30%), abdominal cavity (25%), pelvic lymph node (20%), and extranodal tissue. Can be affected, the most common of which is skin ulcers (30%). At the time of diagnosis, more patients AnnArbor stage III (35%) and IV (35%), and B symptoms (55%) and LDH levels (70%). The whole group was positive for BerH2. The immunophenotype was mainly T cells (60%), and part of B cells (35%) or non-B non-T (5%). Short-term efficacy: Effectiveness (CR+PR) in 17 cases (85%), including complete remission (CR) in 12 cases (60%). The 1-year, 2-year and 3-year survival rates were 54.9%. Whether the first chemotherapy was CR, B symptoms, or giant lesions were all factors affecting survival. Conclusion: ALCL is a non-Hodgkin’s lymph with special immunophenotype and clinical characteristics.