Department of Hematology and Lymphoma Research Center, Peking University, Third Hospital, Beijing, PR 100191, China.
J Hematol Oncol. 2012 Jul 7;5:38. doi: 10.1186/1756-8722-5-38.
Systemic anaplastic large cell lymphoma (S-ALCL) is a rare disease with a highly variable prognosis and no standard chemotherapy regimen. Anaplastic lymphoma kinase (ALK) has been reported as an important prognostic factor correlated with S-ALCL in many but not all studies. In our study, we retrospectively analyzed 92 patients with S-ALCL from the Peking University Lymphoma Center for clinical and molecular prognostic factors to make clear the role of ALK and other prognostic factors in Han Chinese S-ALCL.
The majority of Chinese S-ALCL patients were young male patients (median age 26, male/female ratio 1.7) and the median age was younger than previous reports regardless of ALK expression status. The only statistically significant different clinical characteristic in S-ALCL between ALK positive (ALK+) and ALK negative (ALK-) was age, with a younger median age of 22 for ALK+ compared with 30 for ALK-. However, when pediatric patients (≤ 18) were excluded, there was no age difference between ALK+ and ALK-. The groups did not differ in the proportion of males, those with clinical stage III/IV (49 vs 51%) or those with extranodal disease (53 vs 59%). Of 73 evaluable patients, the 3-year and 5-year survival rates were 60% and 47%, respectively. Univariate analysis showed that three factors: advanced stage III/IV, lack of expression of ALK, and high Ki-67 expression, were associated with treatment failure in patients with S-ALCL. However, ALK expression correlated with improved survival only in patients younger than 14 years, while not in adult patients. In multivariate analysis, only clinical stage was an independent prognostic factor for survival. Expressions of Wilms tumor 1 (WT1) and B-cell lymphoma 2 protein (BCL-2) correlated with the expression of ALK, but they did not have prognostic significance. High Ki-67 expression was also a poor prognostic factor.
Our results show that ALK expression alone is not sufficient to determine the outcome of ALCL and other prognostic factors must be considered. Clinical stage is an independent prognostic factor. Ki-67 expression is a promising prognostic factor.
系统性间变性大细胞淋巴瘤（S-ALCL）是一种罕见疾病，其预后高度可变，且无标准的化疗方案。在许多但并非所有研究中，间变性淋巴瘤激酶（ALK）已被报道为与 S-ALCL 相关的重要预后因素。在本研究中，我们回顾性分析了来自北京大学淋巴瘤中心的 92 例 S-ALCL 患者的临床和分子预后因素，以明确 ALK 及其他预后因素在中国汉族 S-ALCL 中的作用。
大多数中国 S-ALCL 患者为年轻男性（中位年龄 26 岁，男/女比例 1.7），且无论 ALK 表达状态如何，中位年龄均较既往报道年轻。ALK 阳性（ALK+）与 ALK 阴性（ALK-）S-ALCL 患者之间唯一具有统计学显著差异的临床特征是年龄，ALK+患者的中位年龄为 22 岁，而 ALK-患者的中位年龄为 30 岁。然而，当排除儿科患者（≤18 岁）后，ALK+与 ALK-之间的年龄无差异。两组在男性比例、临床分期 III/IV 期（49%比 51%）或结外疾病（53%比 59%）方面无差异。在 73 例可评估患者中，3 年和 5 年的生存率分别为 60%和 47%。单因素分析显示，晚期 III/IV 期、ALK 表达缺失和 Ki-67 高表达是 S-ALCL 患者治疗失败的相关因素。然而，ALK 表达仅与 14 岁以下患者的生存改善相关，而与成年患者无关。多因素分析显示，只有临床分期是生存的独立预后因素。Wilms 肿瘤 1（WT1）和 B 细胞淋巴瘤 2 蛋白（BCL-2）的表达与 ALK 的表达相关，但它们没有预后意义。Ki-67 高表达也是预后不良的因素。
我们的结果表明，ALK 表达本身不足以确定 ALCL 的结局，必须考虑其他预后因素。临床分期是独立的预后因素。Ki-67 表达是一个有前途的预后因素。
