糖皮质激素预防性使用条件下分化综合征的临床特征及高危因素

Analysis on clinical characteristics and risk factors of differentiation syndrome in prophylactic use of glucocorticoids

目的:探讨糖皮质激素预防性使用条件下急性早幼粒细胞白血病患者发生分化综合征的临床特点和高危因素.方法:回顾性分析湖南省人民医院血液科收治的47例初诊APL患者激素预防性使用条件下,经维A酸联合三氧化二砷双诱导治疗继发分化综合征(DS)的临床表现及高危因素.结果:在使用激素预防的情况下,有18例(38.3%)发生DS,其中轻度DS16例(88.9%),重度DS2例(11.1%);DS发生时间为双诱导治疗后2-22d(中位时间8d).发生DS患者中以体重增加最多,12例(66.7%),重度DS患者均出现了肾衰竭.单因素分析结果提示初诊时乳酸脱氢酶(LDH)水平和PML-RARα融合基因分型为发生DS的高危因素.而多因素分析显示PML-RARα融合基因分型为DS的独立危险因素.结论:在糖皮质激素预防性使用条件下,APL患者双诱导治疗中继发DS的发生率仍相对较高,但重度DS的发生率减低.PML-RARα融合基因分型为DS的独立危险因素.

Objective To explore the clinical characteristics and risk factors of differentiation syndrome in acute promyelocytic leukemia(APL) patients who treated with ATRA and AS_(2)O_(3) in prophylactic use of glucocorticoids. Methods Clinical data from 47newly diagnosed APL patients received prophylactic glucocorticoids were retrospectively studied. The clinical characteristics and risk factors for DS were analyzed. Results In prophylaxis usage of glucocorticoids, the total incidence of DS was 38.3%(18/47).Among them, 16 cases(88.9%) had mild DS and 2 cases(11.1%) had severe DS. The onset time of DS was 2-22d after therapy(median time 8d). Weight gain were the most common clinical manifestation. The total incidence of weight gain was 66.7%(12/18).All severe DS patients exhibited renal failure. Univariate analysis showed that the level of lactate dehydrogenase(LDH) and PML-RARα subtype were high risk factors for DS, while multivariate analysis showed that PML-RARα subtype was an independent risk factors for DS. Conclusion Under the condition of prophylactic use of glucocorticoids, the incidence of DS in APL patients was still relatively high, but the incidence of severe DS was decreased. PML-RARα subtype was an independent risk factor for DS.