CAJ | 학술논문

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。
목적 분석원발계통성간변성대세포림파류( ALCL)적림상병리특정화면역조직화학특점，제고진치수평。방법선취22례ALCL환자，균진행분기、국제예후지수(IPI)、유산탈경매(LDH)검측，응용면역조직화학SP법검측간변성림파류격매(ALK)、Ki-67、Caspase-3、CD30、EMA、Granzyme B등，회고성분석환자림상、병리형태학자료、면역표형급생물학특성，병진행예후분석。결과22례균위원발계통성ALCL，ALK+ 15례(68.2％)，ALK-7례(31.8％)；AILK+환자발병년령、Ki-67증식지수교ALK-환자저，Caspase-3표체솔고，차이유통계학의의(x2 =4.618，P= 0.032)；15례ALK+ALCL균표체CD30화EMA。ALCL중ALK적표체여Ki-67、Caspase-3적표체정부상관(r= -0.581,P= 0.006;r=0.458，P=0.032)。ALK+병례교ALK-병례GranzymeB(x2=0.11,P=0.74)、TIA-1( x2= 0.01，P=0.92)적표체솔고，단차이무통계학의의(P＞0.05)。유효솔위54.5％(12/22)，기중완전완해솔위18.2％(4/22)；전조중위생존기12개월，1년생존솔위59.1％( 13/22)，2년생존솔위50.0％(11/22)。Ann Arbor분기、LDH급IPI여질병예후상관。결론ALK+교ALK-ALCL환자핵증식저，악성정도저，림상특정화면역표형구유일정적특정성；ALK、Ki-67、Caspase-3、분기、혈청LDH급IPI대예측ALCL환자적생존화지도치료유방조。
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.