Tchen N, Eghbali H, Soubeyran P, Trojani M, Richaud P, Bonnel C, Quénel-Tueux N, Hoerni B
Service d'hématologie, Institut Bergonié, Bordeaux, France.
Bull Cancer. 1997 Jul;84(7):704-8.
Mediastinal B-cell lymphomas (with or without sclerosis) have been recently recognized as an entity with particular clinical features. We report 26 patients with a mediastinal large B-cell lymphoma. They represent 5% of the patients with aggressive non-Hodgkin's lymphoma and 2% of all non-Hodgkin's lymphoma seen in our centre between 1962 and 1990. They include 19 females (73%) and 7 males (27%). The sex ratio was 2.7 and the median age was 44 years (range: 17-84 years). Compressive symptoms in relation with a bulky mediastinum were present in 21 cases (80%) and with B symptoms in 5 cases. All these patients received 2 to 4 cycles of chemotherapy with a CHOP-like protocol (epirubicin or doxorubicin, cyclophosphamide, vincristine and prednisone) followed in 24 cases by mediastinum irradiation (40 Gy). Two patients progressed during chemotherapy and did not receive radiotherapy. Nineteen patients had a consolidation chemotherapy according to the same protocol. Twenty-one patients achieved a complete remission after chemotherapy or radiotherapy and 5 failed. Two patients relapsed at 10 months and 9 years. Seventeen patients are alive and in first complete remission with a median follow-up of 102 months (range: 60-260 months). Using the Kaplan-Meier method, the overall survival at 5 and 10 years was respectively 77 and 61% and the relapse-free survival was respectively 68 and 57%. These results confirm the previous findings concerning this distinct entity which is characterized by a predilection for young women, compressive symptoms, a slow response to treatment and a rather good prognosis.
纵隔B细胞淋巴瘤(伴或不伴硬化)最近被认为是一种具有特殊临床特征的疾病实体。我们报告了26例纵隔大B细胞淋巴瘤患者。他们占1962年至1990年间我院侵袭性非霍奇金淋巴瘤患者的5%,占所有非霍奇金淋巴瘤患者的2%。其中女性19例(73%),男性7例(27%)。男女比例为2.7,中位年龄为44岁(范围:17 - 84岁)。21例(80%)患者出现与纵隔肿块相关的压迫症状,5例出现B症状。所有这些患者均接受了2至4个周期的类似CHOP方案(表柔比星或多柔比星、环磷酰胺、长春新碱和泼尼松)化疗,24例随后接受纵隔放疗(40 Gy)。2例患者在化疗期间病情进展,未接受放疗。19例患者根据相同方案进行了巩固化疗。21例患者在化疗或放疗后达到完全缓解,5例未缓解。2例患者分别在10个月和9年后复发。17例患者存活且处于首次完全缓解状态,中位随访时间为102个月(范围:60 - 260个月)。采用Kaplan-Meier法,5年和10年的总生存率分别为77%和61%,无复发生存率分别为68%和57%。这些结果证实了先前关于这个独特疾病实体的发现,其特点是好发于年轻女性、有压迫症状、对治疗反应缓慢且预后相当良好。