原发性血小板增多症血清糖酵解酶乳酸脱氢酶及神经元特异性烯醇化酶水平变化的临床观察

目的:探讨原发性血小板增多症(ET)患者血清糖酵解酶乳酸脱氢酶(LDH)和神经元特异性烯醇化酶(NSE)的水平变化及临床意义。方法:采用酶法测定89例ET患者初诊时血清LDH水平,41例采用电化学发光法测定血清NSE水平,并对结果进行统计学分析。结果:89.89%患者血清LDH水平高于正常,均值较对照组明显升高(P<0.01)。87.80%患者血清NSE水平高于正常值,均值较对照组明显升高(P<0.01)。血清LDH及NSE水平均与血小板计数呈正相关(P<0.01或P<0.05)。血清LDH与NSE水平呈正相关(P<0.01)。结论:ET患者血清LDH及NSE水平升高可反映血小板的增殖程度。

Chromosome band 11q23 deletion predicts poor prognosis in bone marrow metastatic neuroblastoma patients without MYCN amplification

Background:Interphase fluorescence in situ hybridization(FISH)of bone marrow cells has been confirmed to be a direct and valid method to assess the v-myc avian myelocytomatosis viral oncogene neuroblastoma derived homolog(MYCN)amplification in patients with bone marrow metastatic neuroblastoma.MYCN amplification alone,however,is insufficient for pretreatment risk stratification.Chromosome band 11q23 deletion has recently been included in the risk stratification of neuroblastoma.In the present study,we aimed to evaluate the biological characteristics and prog-nostic impact of 11q23 deletion and MYCN amplification in patients with bone marrow metastatic neuroblastoma.Methods:We analyzed the MYCN and 11q23 statuses of 101 patients with bone marrow metastatic neuroblastoma using interphase FISH of bone marrow cells.We specifically compared the biological characteristics and prognostic impact of both aberrations.Results:MYCN amplification and 11q23 deletion were seen in 12(11.9%)and 40(39.6%)patients.The two mark-ers were mutually exclusive.MYCN amplification occurred mainly in patients with high lactate dehydrogenase(LDH)and high neuron-specific enolase(NSE)levels(both P<0.001),and MYCN-amplified patients had more events(tumor relapse,progression,or death)than MYCN-normal patients(P=0.004).11q23 deletion was associated only with age(P=0.001).Patients with MYCN amplification had poorer outcomes than those with normal MYCN(3-year event-free survival[EFS]rate:8.3±8.0%vs.43.8±8.5%,P<0.001;3-year overall survival[OS]rate:10.4±9.7%vs.63.5%±5.7%,P<0.001).11q23 deletion reflected a poor prognosis only for patients with normal MYCN(3-year EFS rate:34.3±9.5%vs.53.4±10.3%,P=0.037;3-year OS rate:42.9±10.4%vs.75.9±6.1%,P=0.048).Those with both MYCN amplification and 11q23 deletion had the worst outcome(P<0.001).Conclusions:Chromosome band 11q23 deletion predicts poor prognosis only in bone marrow metastatic neuroblastoma patients without MYCN amplification.Combined assessment of the two markers was much superior to single-marker assessment in recognizing the patients at a high risk of disease progression.