目的 探讨继发性噬血细胞综合征(HPS)成年患者的临床特征及外周血检查指标与疗效的关系.方法 收集2014年4月至2017年3月南京医科大学第一附属医院及南京医科大学附属江宁医院收治的61例成年HPS患者,其中男性38例,女性23例,中位年龄48岁(17～86岁),对其临床表现、实验室检查结果等进行回顾性分析.结果 不同诱因导致的HPS患者间治疗有效率比较,差异无统计学意义(P＝0.184).患者治疗前的凝血酶原时间(PT)长于治疗后[(12.90±1.97)s比(12.35± 1.78)s,P＝0.046],乳酸脱氢酶(LDH)水平高于治疗后(中位数:476 U／L比231 U／L,P＝0.000), D-二聚体(D-D)水平高于治疗后(中位数:1.46 mg／L比0.51 mg／L,P＝0.007),天冬氨酸氨基转移酶(AST)水平高于治疗后(中位数:54.9 U／L比26.0 U／L,P＝0.000),血清钙水平低于治疗后[(2.07± 0.20)mmol／L比(2.18±0.23)mmol／L,P＝0.043].治疗有效组(32例)患者治疗前外周血血小板计数(Plt)高于无效组(29例)(中位数:104.0×109／L比63.5×109／L,P＝0.007),清蛋白(ALB)水平高于无效组[(35.50±6.17)mmol／L比(31.93±6.54)mmol／L,P＝0.033],血清钙水平高于无效组[(2.18±0.18)mmol／L 比(2.08±0.20)mmol／L,P＝0.047],PT 短于无效组[(12.40±1.76)s 比(13.43±2.06)s,P＝0.041],LDH水平低于无效组(中位数:415.0 U／L比593.5 U／L,P＝0.032).结论 成年继发性HPS患者Plt、ALB、血清钙水平均降低,PT延长,D-D、LDH水平升高,可能提示治疗预后不佳,值得进一步关注.%Objective To explore relationship between clinical features and peripheral blood test indicators and curative effect in adult patients with acquired hemophagocytic syndrome (HPS). Methods A total of 61 adult patients with acquired HPS who were admitted to the First Affiliated Hospital with Nanjing Medical University and the Affiliated Jiangning Hospital of Nanjing Medical University from April 2014 to March 2017 were enrolled, including 38 males and 23 females, with a median age of 48 years (17-86 years). The retrospective analyses of their clinical data and laboratory examination results were made in this study. Results There was no significant difference in the therapeutic effective rate of 61 HPS patients caused by different inducements after treatment (P ＝0.184). The prothrombin time (PT) before treatment was higher than that after treatment [(12.90±1.97) s vs. (12.35±1.78) s, P＝ 0.046]; the level of lactate dehydrogenase (LDH) before treatment was higher than that after treatment (median: 476 U/L vs. 231 U/L, P ＝ 0.000); the level of D-dimer (D-D) before treatment was higher than that after treatment (median: 1.46 mg/L vs. 0.51 mg/L, P ＝ 0.007); the level of aspartate aminotransferase (AST) before treatment was higher than that after treatment (median: 54.9 U/L vs. 26.0 U/L, P＝ 0.000); the level of serum calcium before treatment was lower than that after treatment [(2.07±0.20) mmol/L vs. (2.18±0.23) mmol/L, P ＝ 0.043]. The peripheral blood platelet counts (Plt) in the effective group (32 cases) before treatment was higher than that in the ineffective group (29 cases) (median: 104.0×109/L vs. 63.5×109/L, P ＝0.007), the level of albumin (ALB) in the effective group was higher than that in the ineffective group [(35.50 ±6.17) mmol/L vs. (31.93 ±6.54) mmol/L, P ＝ 0.033], the level of serum calcium in the effective group was higher than those in the ineffective group [(2.18±0.18) mmol/L vs. (2.08±0.20) mmol/L, P ＝ 0.047], the level of prothrombin time (PT) in the effective group was lower than that in the ineffective group [(12.40±1.76) s vs. (13.43±2.06) s, P ＝ 0.041], and the level of LDH in the effective group was lower than that in the ineffective group (median: 415.0 U/L vs. 593.5 U/L, P＝ 0.032). Conclusion The lower expressions of Plt, ALB and serum calcium, and the higher expressions of PT and LDH may indicate the poor prognosis of adult acquired HPS, and there fore these patients need to be paid attention.
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