Sissolak Gerhard, Juritz June, Sissolak Dagmar, Wood Lucille, Jacobs Peter
Division of Clinical Haematology - Department of Internal Medicine, Faculty of Health Sciences, Stellenbosch University, South Africa.
Transfus Apher Sci. 2010 Apr;42(2):141-50. doi: 10.1016/j.transci.2010.01.009. Epub 2010 Feb 10.
Substantial geographical differences exist for Hodgkin and other lymphoproliferative disorders with these having previously been documented in a report from the lymphoma reclassification project. In the light of rampant human immunodeficiency syndrome, largely centred in sub-Sahara, this experience is updated in a further 512 consecutive individuals treated over an 8-year period in a privately based academic centre. Median age was 55.2 years 61% were males, 10% had Hodgkin lymphoma and, overall, constitutional symptoms were present in 20%. Prior to referral 19% had received chemotherapy and a further 20% some form of irradiation. Median survival in hairy cell leukaemia (n=14), chronic lymphocytic leukaemia-small lymphocytic lymphoma (n=103), Hodgkin (n=41) and follicular lymphoma (n=59) was not reached at the time of analysis and exceeded 36 months. This was followed by 32 months for those with mantle cell (n=7), splenic (n=2) and extranodal marginal cell (n=11), 24 months for T-cell lymphomas (n=24), 20 months for diffuse large B-cell variants (n=88) but only 12 months for the aggressive tumours exemplified by Burkitt (n=7) and lymphoblastic subtypes (n=6). The remaining 36 patients had to be excluded because numbers were too small for statistical analysis or unreliable staging. Adverse factors were constitutional symptoms, prior treatment with chemotherapy, intermediate or high-risk scores as defined by the international prognostic index, histologic grading and certain anatomical sites of primary tumour. In contrast gender, staging by Rye or Rai classification, retroviral infection and prior treatment with radiotherapy were without effect. Overall survival at 3 years in each category was compared to the curve for the entire cohort and was 100% in hairy cell leukaemia receiving two chlorodeoxyadenosine and greater than 88% in Hodgkin lymphoma treated according to the German study group protocols (p=0.0004). Corresponding figures for chronic lymphocytic leukaemia-small lymphocytic lymphoma were 82% (p=0.0006), follicular lymphoma 71% (p=0.060), peripheral T-cell lymphoma 43% (p=0.0156), diffuse large B-cell lymphoma 39% (p<0.0001), aggressive tumours 25% (p=0.0002) and for the indolent categories including mantle cell, splenic and extra nodal marginal cell lymphomas 22% (p=0.2023). Outcome argues in favour of patient management by a multidisciplinary team implicit in which are standardised protocols for diagnosis, staging and treatment. Under these circumstances the well recognized centre effect applies when results approximate those from first world reference centres. Conversely any deviation from such a disciplined approach is unlikely to achieve comparable benefit and therefore to be strongly discouraged.
霍奇金淋巴瘤和其他淋巴增生性疾病存在显著的地域差异,淋巴瘤重新分类项目的一份报告中曾对此有过记载。鉴于以撒哈拉以南地区为主要发病中心的人类免疫缺陷综合征肆虐,在一家私立学术中心对连续512例患者进行了为期8年的治疗,从而更新了这方面的经验。患者中位年龄为55.2岁,61%为男性,10%患有霍奇金淋巴瘤,总体而言,20%的患者有全身症状。转诊前,19%的患者接受过化疗,另有20%接受过某种形式的放疗。分析时,毛细胞白血病(n = 14)、慢性淋巴细胞白血病 - 小淋巴细胞淋巴瘤(n = 103)、霍奇金淋巴瘤(n = 41)和滤泡性淋巴瘤(n = 59)的中位生存期未达到,且超过36个月。其次,套细胞淋巴瘤(n = 7)、脾淋巴瘤(n = 2)和结外边缘细胞淋巴瘤(n = 11)患者的生存期为32个月,T细胞淋巴瘤(n = 24)患者为24个月,弥漫性大B细胞淋巴瘤变异型(n = 88)患者为20个月,但以伯基特淋巴瘤(n = 7)和淋巴母细胞亚型(n = 6)为代表的侵袭性肿瘤患者仅为12个月。其余36例患者因数量过少无法进行统计分析或分期不可靠而被排除。不良因素包括全身症状、先前接受化疗、国际预后指数定义的中危或高危评分、组织学分级以及原发性肿瘤的某些解剖部位。相比之下,性别、Rye或Rai分期、逆转录病毒感染以及先前接受放疗则无影响。将各分类中3年的总生存率与整个队列的曲线进行比较,接受两种氯脱氧腺苷治疗的毛细胞白血病患者3年总生存率为100%,按照德国研究组方案治疗的霍奇金淋巴瘤患者大于88%(p = 0.0004)。慢性淋巴细胞白血病 - 小淋巴细胞淋巴瘤的相应数据为82%(p = 0.0006),滤泡性淋巴瘤为71%(p = 0.060),外周T细胞淋巴瘤为43%(p = 0.0156),弥漫性大B细胞淋巴瘤为39%(p < 0.0001),侵袭性肿瘤为25%(p = 0.0002),包括套细胞淋巴瘤、脾淋巴瘤和结外边缘细胞淋巴瘤在内的惰性淋巴瘤类别为22%(p = 0.2023)。结果表明,由多学科团队进行患者管理是有益的,其中隐含着诊断、分期和治疗的标准化方案。在这种情况下,当结果接近第一世界参考中心的结果时,公认的中心效应就会起作用。相反,任何偏离这种规范方法的做法都不太可能获得类似的益处,因此应强烈反对。