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背景与目的:原发系统型间变大细胞性淋巴瘤(anaplastic large cell lymphoma,ALCL)的临床特征和疾病转归国内外报道的结果存在一定的差异。间变性淋巴瘤激酶蛋白(anaplastic lymphoma kinase,ALK)被认为是原发系统型ALCL较为特异的标志,表达与否和患者的临床特征及预后相关。本研究目的是探讨原发系统型ALCL患者的临床特点和预后,检测ALK的表达并探讨其临床意义。方法:回顾性分析1997年1月至2006年6月中山大学肿瘤防治中心收治的57例原发系统型ALCL患者的临床资料。采用免疫组化SP法检测肿瘤标本中ALK的表达。结果:57例患者的中位年龄为30岁,男女比例为1.9∶1,有B症状、Ⅲ～Ⅳ期和结外侵犯患者分别占57.9%、40.4%和40.4%。所有患者接受化疗,56例可评价疗效,40例(71.4%)取得完全缓解,客观有效率为96.4%;中位随访49.1个月,中位无疾病进展生存期为35.2个月,预计5年生存率为65.2%;国际预后指数评分低/低中危患者和中高/高危患者的预计5年生存率分别为68.9%和33.3%(P=0.010)。46例中ALK阳性29例(63%),发病年龄小于阴性者(P=0.001),预计5年生存率优于阴性者(P<0.01)。多因素分析显示,ALK表达状况、原发部位和乳酸脱氢酶水平是ALCL患者的独立预后因素。结论:原发系统型ALCL发病年龄较年轻,化疗有效率高,预后良好,但ALK阴性、IPI中高/高危、尤其合并结外侵犯和乳酸脱氢酶水平升高者预后较差。
BACKGROUND & OBJECTIVE: The clinical characteristics and disease outcome of primary systemic anaplastic large cell lymphoma (ALCL) are different at home and abroad. Anaplastic lymphoma kinase (ALK) is considered as a more specific marker of primary systemic ALCL. The expression of ALK is associated with the clinical characteristics and prognosis of patients. The purpose of this study was to investigate the clinical features and prognosis of patients with primary systemic ALCL and to detect the expression of ALK and to explore its clinical significance. Methods: The clinical data of 57 patients with primary systemic ALCL admitted from Sun Yat-sen University Cancer Center from January 1997 to June 2006 were retrospectively analyzed. Immunohistochemical SP method was used to detect the expression of ALK in tumor samples. Results: The median age of 57 patients was 30 years old, the ratio of male to female was 1.9: 1. The patients with B symptoms accounted for 57.9%, 40.4% and 40.4% of patients with stage Ⅲ-Ⅳ and extranodal invasion respectively. All of the patients underwent chemotherapy. Fifty-six patients (71.4%) achieved complete remission. The objective response rate was 96.4%. The median follow-up was 49.1 months. The median progression-free survival was 35.2 months. The estimated 5-year The survival rate was 65.2%. The estimated 5-year survival rates of patients with low / medium-risk and high-risk / high-risk patients with international prognostic index were 68.9% and 33.3%, respectively (P = 0.010). Among the 46 cases, ALK positive was found in 29 cases (63%) and the age of onset was lower than that in the negative (P = 0.001). The 5-year survival rate was expected to be superior to the negative (P <0.01). Multivariate analysis showed that ALK expression, primary site and lactate dehydrogenase level were independent prognostic factors in patients with ALCL. Conclusions: The age of onset of primary systemic ALCL is younger, the chemotherapy is more effective and the prognosis is good. However, ALK is negative, and the high / high risk of IPI, especially in combination with extranodal invasion and elevated lactate dehydrogenase, have a poor prognosis.