不同年龄组儿童噬血细胞综合征的临床特点及预后研究

目的 探讨不同年龄组儿童噬血细胞综合征(HPS)的临床特点与预后因素分析.方法 将85例HPS患儿按年龄分为0～2岁(A组)、＞2～8岁(B组)和＞8～14岁(C组),从病因、病理、临床症状、实验室检查及预后等方面进行回顾性分析.结果 A、B和C组病因均以EB病毒感染为主,感染阳性率C组明显高于A、B组;骨髓检查粒细胞红细胞比例(粒红比)小于1.0的发生率,A组高于C组;颈部淋巴结肿大发生在B组中多见,肝脾肿大在C组中最少见;浆膜腔积液发生在B组多见;实验室检查乳酸脱氢酶(LDH)B组高于A组,以上差异均有统计学意义(P＜0.05).其他临床症状和实验室检查结果在组之间差异无统计学意义(P＞0.05).存活与死亡患儿比较,浆膜腔积液、三酰甘油(TG)、CD4/CD8及骨髓检查粒红比比较,差异有统计学意义(P＜0.05),将上述因素进行Logistic分析,显示TG＞3.5 mmol/L、CD4/CD8＜ 1.24、骨髓粒红比小于1.02是影响儿童HPS预后的危险因素(P＜0.05).结论 儿童HPS有部分临床特点与发病年龄有关,TG、CD4/CD8及骨髓粒红比水平是影响预后的危险因素.

Characteristics and prognosis of child hemophagocytic syndrome in different age groups

Objective To explore the clinical characteristics and prognosis factors of child hemophagocytic syndrome(HPS)in different age groups.Methods Eighty-five children cases of HPS were divided into 3 age groups,0-2 years old (group A),＞2-8 years old (group B) and ＞8-14 years old (group C).The etiology,pathology,clinical symptoms,laboratory detection and prognosis were retrospectively analyzed.Results In the etiology,the etiology in the group A,B and C was dominated by EB virus infection,the infection positive rate in the group C was significantly higher than that in the group B and C;in the bone marrow examination:the occurrence rate of granulocytes to erythrocytes ratio ＜1.0 in the group A was higher than that in the group C;in the clinical symptoms,cervical lymph node enlargement mostly occurred in the group B,hepatosplenomegaly was most rare in the group C,and the serous cavity effusion commonly occurred in the group B;in the laboratory detection,the LDH level in the group B was higher than that in the group A,the above differences were statistically significant(P＜0.05).There were no statistically significant differences in other clinical symptoms and laboratory detection results among the three groups (P＞0.05);In the comparison between the survival group and death group,the serous cavity effusion,triglyceride(TG),CD4/CD8 ratio and granulocytes to erythrocytes ratio in bone marrow had statistical difference(P＜0.05).The above factors were performed the Logistic analysis,the results showed that TG＞3.5 mmol/L,CD4/CD8 ratio ＜1.24 and bone marrow granulocytes to erythrocytes ratio ＜1.02 were the unfavorable risk factors affecting the prognosis of child HPS (P＜0.05).Conclusion Partial clinical characteristics of child HPS are related with the onset age,TG level,CD4/CD8 ratio and bone marrow granulocytes to erythrocytes ratio are the risk factors affecting prognosis.