Haioun C, Gaulard P, Roudot-Thoraval F, Divine M, Jouault H, Lebourgeois J P, Kuentz M, Farcet J P, Reyes F
Service d'Hematologie Clinique, Hôpital Henri Mondor, Creteil, France.
Am J Clin Oncol. 1989 Oct;12(5):425-9. doi: 10.1097/00000421-198910000-00013.
Twenty patients (17 women, three men) with mediastinal diffuse large-cell lymphoma with sclerosis are reported. At the time of diagnosis, the disease was confined to supradiaphragmatic areas in all patients but two, who had kidney involvement (seven were stage I, 11 were stage II, and two were stage IV). A B-cell phenotype was demonstrated in nine of the 11 cases that were analyzed for cell lineage. All patients received an anthracyclin-containing regimen followed by mantle radiotherapy. Analysis of pretreatment characteristics showed that only the extent of disease influences outcome. Moreover, achievement of complete remission (CR) after chemotherapy appears to be a major prognostic factor. Two-year survival was better in patients who reached CR after chemotherapy than in those who did not (100% versus 9%; p less than 0.0001). Overall 2-year and 7-year survival rates were 50% and 33%, respectively. Therefore, localized mediastinal large cell lymphoma with sclerosis should be considered a high-risk subtype of non-Hodgkin's lymphoma in which standard treatment approaches are unsuccessful.
本文报告了20例伴有硬化的纵隔弥漫大B细胞淋巴瘤患者（17例女性，3例男性）。诊断时，除2例有肾脏受累外，所有患者病变均局限于膈上区域（7例为I期，11例为II期，2例为IV期）。在11例进行细胞谱系分析的病例中，9例显示为B细胞表型。所有患者均接受含蒽环类药物的化疗方案，随后进行斗篷野放疗。对治疗前特征的分析表明，只有疾病范围影响预后。此外，化疗后达到完全缓解（CR）似乎是一个主要的预后因素。化疗后达到CR的患者2年生存率高于未达到CR的患者（100%对9%；P<0.0001）。总体2年和7年生存率分别为50%和33%。因此，伴有硬化的局限性纵隔大细胞淋巴瘤应被视为非霍奇金淋巴瘤的高危亚型，采用标准治疗方法效果不佳。
