噬血细胞形态在感染相关HLH中的临床意义探讨  

Clinical values of morphology of hemophagocytes in infection associated hemophagocytic lymphohistiocytosis

作　　者：陈佳宁[1] 李岩[1] 金爽 徐小用[1] 吴林[3] 车轶群 Chen Jianing;Li Yan;Jin Shuang;Xu Xiaoyong;Wu Lin;Che Yiqun(Department of Clinical Laboratory Center,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China;Department of Clinical Laboratory,Health Service Center,University of Science and Technology Beijing,Beijing 100083,China;Department of Hematology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院临床检验中心,北京100050 [2]北京科技大学社区卫生服务中心检验科,北京100083 [3]首都医科大学附属北京友谊医院血液科,北京100050

出　　处：《中华检验医学杂志》2023年第5期501-509,共9页Chinese Journal of Laboratory Medicine

摘　　要：目的研究感染相关噬血细胞性淋巴组织细胞增生症(IAHLH)患者骨髓涂片噬血细胞(HPC)形态不同, 其临床和实验室特征、细胞因子水平、疾病预后是否有差异。方法回顾性分析2016年12月1日至2021年12月31日首都医科大学附属北京友谊医院初治IAHLH共24例, 选择非噬血细胞性淋巴组织细胞增多症(HLH)感染性疾病20例为对照组。IAHLH组年龄(34±13)岁, 男17例(71%), 女7例(29%);非HLH组年龄(43±16)岁, 男14例(70%), 女6例(30%)。复检初诊骨髓涂片, 将HPC分为不吞噬有核细胞的HPC-1型和吞噬有核细胞的HPC-2型。比较IAHLH组和非HLH组、骨髓有HPC和无HPC、骨髓仅见HPC-1和骨髓可见HPC-2的患者确诊后1年内病死率、HLH-2004诊断相关临床和实验室指标及细胞因子白细胞介素(IL)-6、IL-10、IL-18、干扰素(IFN)-γ水平的组间差异。计数资料两组间比较采用χ^(2)检验和Fisher精确概率检验。非正态分布的计量资料两组间比较采用Mann-WhitneyU检验, 正态分布的计量资料两组间比较采用独立样本t检验。结果发热、肝大和脾大阳性率、病死率在IAHLH组依次为100%(24/24)、63%(15/24)、92%(22/24)、46%(11/24), 非HLH组依次为55%(11/20)、0(0/20)、25%(5/20)、0(0/20), 组间差异均有统计学意义(P<0.01), 在骨髓有无HPC、HPC是否吞噬有核细胞的IAHLH患者间差异均无统计学意义(P>0.05)。骨髓可见HPC-2和骨髓仅见HPC-1的IAHLH患者的IFN-γ水平分别为400(246, 532)、146(38, 180)ng/L, 差异有统计学意义, 前者高于后者[P=0.02, 95%CI 233(75.8~397)], 其他细胞因子和实验室指标差异无统计学意义(P>0.05)。结论 HPC是否吞噬有核细胞, IAHLH患者的主要临床表现和病死率无差异, 但巨噬细胞激活因子IFN-γ水平有差异, HPC吞噬有核细胞者其IFN-γ水平更高。Objective To study the morphology of hemophagocytosis(HPC)in bone marrow smears of patients with infection-associated hemophagocytic lymphohistiocytosis(IAHLH),and further analyse if there were differences in the clinical and laboratory features,the cytokines level and prognosis Methods 24 patients newly diagnosed with IAHLH from 2016-Dec-1 to 2021-Dec-31 in Beijing Friendship Hospital were included as study group,and 20 patients with infectious disease as non-HLH control group.In IAHLH group,mean age was 34±13 years,including 17(71%)males and7(29%)females.In Non-HLH group,mean agewas 43±16 years,including 14(70%)males and6(30%)females.Depending on re-checking phagocytic cell type on the initial bone marrow smear,the HPCs were divided into HPC-1,phagocytizing non-nucleated cells(mature erythrocyte or platelets),and HPC-2,phagocytizing nucleated cells.The differences in clinical presentations covered in HLH-2004 criteria,cytokines value(IL-6,IL-10,IL-18,IFN-γ)recommended in HLH-2022-China guideline,and the mortality within 1 year of diagnosis,were compared between IAHLH and non-HLH groups,between patients with or without HPC,and between patients with HPC-2 or only with HPC-1.For categorical variables,two groups were compared with the use of either the chi-square test or Fisher′s exact test.For non-normal distribution continuous variables,the difference between two groups variation was performed by using Mann-Whitney U test,and for normal distribution continuous variables,the difference was by the Independent Samples t-test.Results The positive rates of fever,hepatomegaly and splenomegalyand the motrtality in IAHLH were 100%(24/24),63%(15/24),92%(22/24)and 46%(11/24),respectivelyin non-HLH were 55%(11/20),0(0/20),25%(5/20),0(0/20),and the differences between two groups were all statistically significant(P<0.01),but thedifferences between groups with and without HPC and between IAHLH patients with HPC-2 or only with HPC-1 were no statistically significanlly,(P>0.05).In IAHLH group,IFN-γin patients with HPC-2 w

关 键 词：噬血细胞 干扰素-Γ 白细胞介素 噬血细胞性淋巴组织细胞增生症 骨髓穿刺 细胞形态学 

分 类 号：R551.2[医药卫生—血液循环系统疾病]