原发系统性间变性大细胞淋巴瘤临床病理特征及免疫表型分析

目的 分析原发系统性间变性大细胞淋巴瘤( ALCL)的临床病理特征和免疫组织化学特点，提高诊治水平。方法选取22例ALCL患者，均进行分期、国际预后指数(IPI)、乳酸脱氢酶(LDH)检测，应用免疫组织化学SP法检测间变性淋巴瘤激酶(ALK)、Ki-67、Caspase-3、CD30、EMA、Granzyme B等，回顾性分析患者临床、病理形态学资料、免疫表型及生物学特性，并进行预后分析。结果22例均为原发系统性ALCL，ALK+ 15例(68.2％)，ALK-7例(31.8％)；AILK+患者发病年龄、Ki-67增殖指数较ALK-患者低，Caspase-3表达率高，差异有统计学意义(x2 =4.618，P= 0.032)；15例ALK+ALCL均表达CD30和EMA。ALCL中ALK的表达与Ki-67、Caspase-3的表达呈负相关(r= -0.581,P= 0.006;r=0.458，P=0.032)。ALK+病例较ALK-病例GranzymeB(x2=0.11,P=0.74)、TIA-1( x2= 0.01，P=0.92)的表达率高，但差异无统计学意义(P＞0.05)。有效率为54.5％(12/22)，其中完全缓解率为18.2％(4/22)；全组中位生存期12个月，1年生存率为59.1％( 13/22)，2年生存率为50.0％(11/22)。Ann Arbor分期、LDH及IPI与疾病预后相关。结论ALK+较ALK-ALCL患者核增殖低，恶性程度低，临床特征和免疫表型具有一定的特征性；ALK、Ki-67、Caspase-3、分期、血清LDH及IPI对预测ALCL患者的生存和指导治疗有帮助。

Analysis of the clinicopathological characteristics and immunophenotypic of primary systemic anaplastic large cell lymphoma

Objective To Analysis the clinicopathology characteristics, immunophenotype of anaplastic large cell lymphoma (ALCL) in order to improve its diagnostic and therapeutic accuracy. Methods The expression of ALK, Ki-67, Caspase-3, CD2, CD3, CD4, CD5, CD7, CD20, CD15, CD30, EMA, Granzyme B,Perforation element and TIA-1 was detected in 22 cases ALCL using Immunohistochemical S-P method. The clinical, immunophenotypic and histopathologic features of 22 cases ALCL were retrospectively studied. Results All 22 cases were primary systemic ALCL. Among them, 15 cases were ALK-positive and 7 cases were ALK- negative. The patients with ALK-positive were younger than those with ALK-negative case (P ＜0.05). Immunohistochemical study showed that ALK-positive ALCL always expressed CD30 and EMA. In ALK-positive ALCL cases, the ageswas younger and the expressions of Ki-67 were lower, and the expressions of caspase-3 were higher than those of ALK-negative cases, in which the difference was statistically significant (P ＜0.05). The expression of Ki-67 was negative correlated with caspase-3 (P ＜0.05). Among the 22 cases of ALCL, 4 patients (18.2 ％) achieved a complete remission (CR) and 8 patients (36.4 ％) achieved a part remission (PR), and the rate of CR and PR was 54.5 ％ (12/22). The 1-year overall survival rate for all patients were 59.1％ (13/22). ALK, Ki-67, Caspase-3, clinical staging,LDH level and IPI score were found to be the prognostic factors associated with overall survival in ALCL. Conclusion ALCL cases with positive for ALK showed low degree of malignancy than ALCL cases with negative for ALK. The differences of clinical features, immunophenotypes between ALK-positive ALCL and ALK-negative ALCL groups are helpful in the differential diagnosis. The prognosis of ALCL is significantly correlated with ALK, Ki-67, Caspase-3, stages, level of LDH and IPI score.