57例原发系统型间变大细胞性淋巴瘤临床分析

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（尸〈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。

Clinical analysis of primary systemic anaplastic large cell lymphoma:a report of 57 cases

Background and Objective： The clinical characteristics and prognosis of primary systemic anaplastic large cell lymphoma （ALCL） are various according to different reports. Anaplastic lymphoma kinase （ALK）, a specific marker of primary systemic ALCL, is related with its clinical characteristics and prognosis. This study was to investigate the clinical characteristics and prognosis of primary systemic ALCL, and to explore the expression and clinical significance of ALK. Methods. Clinical data of 57 primary systemic ALCL patients, treated in Cancer Center of Sun Yat-sen University from January 1997 to Junuary 2006, were reviewed. The expression of ALK in 46 tumor specimens was detected by SP immunohisochemistry. Results： The median age of the 57 patients was 30. The male/female ratio was 1.9：1. Of the 57 patients, 33 （57.9%） had B symptoms, 23 （40.4%） had Ann Arbor stage Ⅲ-Ⅳ tumors, and 23 （40.4%） had extranodal disease at diagnosis. All patients received chemotherapy. Of the 56 evaluable patients, 40 （71.4%） achieved complete remission. The overall response rate was 96.4%. With a median follow-up of 49.1 months, the median time to progression was 35.2 months and the expected 5-year survival rate was 65.2%. The expected 5-year survival rate was significantly higher in low/low- intermediate International Prognostic Index （IPI） risk group than in high- intermediate/high IPI risk group （68.9% vs. 33.3%, P=0.010）. The positive rate of ALK was 63.0% in the 46 cases detected. Compared with ALK-negative patients, ALK-positive patients were younger （P=0.001） and had higher expected 5-year survival rate （P〈0.01）. Multivariate analysis confirmed the independent prognostic values of ALK expression, primary disease site and lactate dehydrogenase （LDH） level. Conclusions. Primary systemic ALCL usually occurs in young patients, with good response to chemotherapy and good prognosis. No ALK expression, high-intermediate/high IPI, extranodal disease and elevated LDH level are co