不同年龄组儿童噬血细胞综合征的临床特点及预后研究

Characteristics and prognosis of child hemophagocytic syndrome in different age groups

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>3.5mmol/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。

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 granutocytes 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.