急性白血病患者蛋白C系统检测的临床意义

Clinical significance of measurement of protein C system in patients with acute leukemia

目的探讨急性白血病（ＡＬ）患者蛋白Ｃ（ＰＣ）系统的改变及其与分型、出血情况和预后的关系。方法运用ＥＬＩＳＡ或发色底物法对９３例ＡＬ患者血浆ＰＣ活性和抗原（ＰＣ：Ａ和ＰＣ：Ａｇ）、血栓调节蛋白（ＴＭ）及蛋白Ｓ（ＰＳ）水平进行了检测。结果治疗前血浆ＴＭ水平显著升高，ＰＣ：Ａｇ水平低于正常，ＰＣ：Ａ和ＰＳ水平与正常对照组无显著差异；缓解后除ＰＣ：Ａ和急性淋巴细胞白血病患者ＰＣ：Ａｇ增高外余均恢复至正常范围内。上述指标与出血程度无关。全反式维甲酸治疗组ＰＣ：Ａ和ＴＭ有所升高，在三氧化二砷治疗组未发现上述现象。治疗前后ＴＭ升高，治疗前ＰＳ降低者预后较差，其中治疗前ＰＳ和治疗后ＴＭ是决定患者无复发生存时间独立的预后因素，治疗后ＴＭ是决定患者总生存时间独立的预后因素。结论ＡＬ发病过程中存在ＰＣ系统的激活，并随病情的好转而基本改善。ＰＣ系统并非是决定出血程度的关键因素，但ＴＭ升高及ＰＳ消耗与患者的预后密切相关。

Objective To study the variation of protein C (PC) system in acute leukemia (AL) patients and their relations to FAB classification, bleeding symptom and prognosis. Methods The activity and antigen level of PC (PC:A and PC:Ag), as well as thrombomodulin (TM) and Protein S (PS) were measured among 93 cases of AL using ELISA or chromogenic substrate assay. Results Plasma level of TM was elevated significantly at diagnosis; while, PC:Ag was decreased, and PC:A and PS remained unchanged. All the above variables returned to normal after complete remission (CR) except the increase of PC:A and PC:Ag in acute lymphocytic leukemia. None of them was related to hemorrhagic manifestation. All-trans retinoic acid enhanced the level of PC:A and TM, which was not found in arsenic trioxide group in acute promyelocytic leukemia. Increased TM be- fore treatment and after CR, decreased PS before treatment were correlated with poor prognosis. PS before treatment and TM after CR were independent prognostic factors considering relapse-free survival. TM after CR was independently prognostic factors of overall survival. Conclusion There existed the activation of PC system in AL, which could be greatly improved after CR. Although the activation of PC system was not the detendnative factors considering the hemorrhagic manifestation, the elevation of TM, as well as the consumption of PS proved to be an indicator of poor prognosis in AL patients.