青年、成年和老年慢性髓系白血病患者之间的差异。

Differences among young adults, adults and elderly chronic myeloid leukemia patients.

机构信息

Department of Experimental, Diagnostic and Specialty Medicine, Institute of Hematology 'L. and A. Seràgnoli', 'S. Orsola-Malpighi' University Hospital.

出版信息

Ann Oncol. 2015 Jan;26(1):185-192. doi: 10.1093/annonc/mdu490. Epub 2014 Oct 30.

DOI:10.1093/annonc/mdu490

PMID:25361995

Abstract

BACKGROUND

The incidence of chronic myeloid leukemia (CML) increases with age, but it is unclear how the characteristics of the disease vary with age. In children, where CML is very rare, it presents with more aggressive features, including huge splenomegaly, higher cell count and higher blast cell percentage.

PATIENTS AND METHODS

To investigate if after childhood the disease maintains or loses these characteristics of aggressiveness, we analyzed 2784 adult patients, at least 18 years old, registered by GIMEMA CML WP over a 40-year period.

RESULTS

Young adults (YAs: 18-29 years old) significantly differed from adults (30-59 years old) and elderly patients (at least 60 years old) particularly for the frequency of splenomegaly (71%, 63% and 55%, P < 0.001), and the greater spleen size (median value: 4.5, 3.0 and 1.0 cm, P < 0.001). According to the EUTOS score, that is age-independent, high-risk patients were more frequent among YAs, than among adult and elderly patients (18%, 9% and 6%, P < 0.001). In tyrosine kinase inhibitors-treated patients, the rates of complete cytogenetic and major molecular response were lower in YAs, and the probability of transformation was higher (16%, 5% and 7%, P = 0.011).

CONCLUSIONS

The characteristics of CML or the host response to leukemia differ with age. The knowledge of these differences and of their causes may help to refine the treatment and to improve the outcome.

CLINICAL TRIAL NUMBERS

NCT00510926, NCT00514488, NCT00769327, NCT00481052.

摘要

背景

慢性髓性白血病（CML）的发病率随年龄增长而增加，但目前尚不清楚该疾病的特征随年龄变化的情况。在儿童中，CML 非常罕见，其表现出更具侵袭性的特征，包括巨大的脾肿大、更高的细胞计数和更高的原始细胞比例。

患者和方法

为了研究儿童期后疾病是否保持或失去这些侵袭性特征，我们分析了 GIMEMA CML WP 在 40 年期间登记的 2784 例至少 18 岁的成年患者。

结果

年轻成人（18-29 岁）与成年患者（30-59 岁）和老年患者（至少 60 岁）显著不同，特别是脾肿大的频率（71%、63%和 55%，P<0.001）和更大的脾脏大小（中位数：4.5、3.0 和 1.0 cm，P<0.001）。根据与年龄无关的 EUTOS 评分，年轻成人患者中高危患者更为常见，而成年和老年患者中高危患者较少（18%、9%和 6%，P<0.001）。在接受酪氨酸激酶抑制剂治疗的患者中，年轻成人患者完全细胞遗传学和主要分子反应的比例较低，转化的概率更高（16%、5%和 7%，P=0.011）。