Clinical characteristics and outcome of young chronic lymphocytic leukemia patients: A single institution study of 204 cases
Clinical characteristics and outcome of young chronic lymphocytic leukemia patients: A single institution study of 204 cases
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DOI：
10.1182/blood.v94.2.448.414k13_448_454
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发表时间：
1999-07-15
期刊：
影响因子：
20.3
通讯作者：
Mandelli, F
中科院分区：
文献类型：
--
作者：
Mauro, FR;Foa, R;Mandelli, F
回顾性分析慢性淋巴细胞白血病（CLL）患者小于或等于55岁的观察在一个单一的机构进行的临床特征和结果的年轻CLL和识别不同的预后特征的患者的目的。在1984年至1994年期间，观察了1，011名CLL患者（204名[20%]小于或等于55岁，807名[80%] >55岁）。在诊断时，年轻和老年患者显示出相似的临床特征分布，除了年轻患者的男性/女性比例显著较高（2.85 v1.29; P <0.0001）。两组患者的第二原发性癌症发病率均较高（8.3%比10.7%），而年轻患者的里希特综合征发病率明显较高（5.9%比1.2%; P < .00001）。年轻和老年患者显示出相似的总体中位生存概率（10年），但其特征在于死亡原因的不同分布：CLL无关死亡和第二原发恶性肿瘤在老年组中占主导地位，而白血病的直接影响在年轻组中普遍存在。虽然年轻和老年患者的生存率相似，但相对生存率的评估显示，该疾病对年轻人群的预期生存概率有更大的不利影响。多变量分析显示，对于年轻CLL患者，只有动态参数，如淋巴细胞倍增时间和其他活动性疾病的体征，是显著影响生存概率的独立因素（P = .00001）。一个长期的临床血液学随访使我们能够确定两个亚组的年轻CLL患者具有不同的预后结果：一组患者（40%）长期稳定的疾病，没有治疗和精算生存概率为94%，在12年诊断和另一组（60%）进行性疾病和治疗后的中位生存概率为5年。对于后者患者，需要在前瞻性、比较性临床试验中研究具有治愈意图的创新疗法的治疗效果。(C)1999年，美国血液学会。
A retrospective analysis on chronic lymphocytic leukemia (CLL) patients less than or equal to 55 years observed at a single institution was performed with the purpose of characterizing the clinical features and outcome of young CLL and of identifying patients with different prognostic features. Over the period from 1984 to 1994, 1,011 CLL patients (204 [20%] less than or equal to 55 years of age and 807 [80%] >55 years of age) were observed. At diagnosis, younger and older patients displayed a similar distribution of clinical features, except for a significantly higher male/female ratio in younger patients (2.85 v 1.29; P < .0001). Both groups showed an elevated rate of second primary cancers (8.3% v 10.7%), whereas the occurrence of Richter's syndrome was significantly higher in younger patients (5.9% v 1.2%; P < .00001). Younger and older patients showed a similar overall median survival probability (10 years) but were characterized by a different distribution of causes of deaths: CLL unrelated deaths and second primary malignancies predominated in the older age group, whereas the direct effects of leukemia were prevalent in the younger age group. Although younger and older patients displayed a similar survival, the evaluation of the relative survival rates showed that the disease had a greater adverse effect on the expected survival probability of the younger population. Multivariate analysis showed that for young CLL patients only dynamic parameters, such as lymphocyte doubling time and other signs of active disease, were the independent factors that significantly influenced survival probability (P = .00001). A prolonged clinico-hematologic follow-up allowed us to identify two subsets of young CLL patients with a different prognostic outcome: a group of patients (40%) with long-lasting stable disease without treatment and an actuarial survival probability of 94% at 12 years from diagnosis and another group (60%) with progressive disease and a median survival probability of 5 years after therapy. For the latter patients, the therapeutic effect of innovative therapies with curative intents needs to be investigated in prospective, comparative clinical trials. (C) 1999 by The American Society of Hematology.