不同年龄亨-舒综合征患者临床与免疫学特征研究  

Comparison of age-based clinical and abnormal immune parameters in patients with Henoch- Schoenlein purpura

作　　者：张娜[1] 郭培京[1] 刘品莉[1] 杨红蓉[1] 肖晶[1] 李新鹏[1] 黄金波 郑以州 

机构地区：[1]山西省中医院血液科,太原030012 [2]中国医学科学院、北京防和医学院血液学研究所、血液病医院

出　　处：《中华血液学杂志》2017年第1期60-64,共5页Chinese Journal of Hematology

摘　　要：目的分析不同年龄亨-舒综合征（HSP）患者临床及免疫学特征，并探讨免疫指标与器官累及的相关性。方法回顾性分析502例HSP患者临床及免疫学特征，比较不同年龄组临床和免疫学特征的差异。结果502例患者中345例（68.73%）首发年龄≤14岁。HSP患者急性期免疫紊乱主要表现为：红细胞沉降率（ESR）增快（41.5%，146/352）、CD3＋细胞比例降低（62.9%，151/240）、CD4＋细胞比例降低（48.3%，116/240）、CD4＋/CD8＋细胞比例升高（24.2%，58/240）、CD3-CD16＋CD56＋细胞比例失衡（21.7%，51/235）、IgA升高（19.7%，14/71）、IgG降低（54.9%，50/91）、IgM异常（13.6%，8/59）、IgE异常（60.2%，239/397）、补体C3降低（27.0%，17/63）、补体C4降低（7.1%，4/56）和抗链球菌溶血素O（ASO）升高（29.1%，111/381）。较之〉14岁组患者，≤14岁组患者首发症状累及关节更为常见（20.8%对7.6%，χ2=13.547，P〈0.001），消化道受累（57.4%对33.8%，χ2=24.106，P〈0.001）和关节受累（55.9%对32.5%，χ2=23.768，P〈0.001）更常见，而肾脏受累较少（21.4%对51.3%，χ2=42.440，P〈0.001）；更多呈现CD3＋、CD4＋细胞比例降低，IgG降低和ASO升高（33.1%对20.0%，χ2=6.656，P=0.010），而IgA升高相对少见（2.6%对39.4%，χ2=15.582，P〈0.001）。上述异常免疫学指标与HSP器官累及存在相关性，IgG水平降低（P〈0.001）和IgE水平升高（P=0.001）的患者常累及消化道，CD3＋CD4＋细胞比例降低（P=0.004）和IgM水平正常（P=0.003）的患者倾向累及关节，而CD3＋细胞比例正常（P=0.014）和CD3＋CD8＋细胞比例升高（P=0.032）的患者肾脏累及较为多见。结论HSP患者急性期存在显著的免疫紊乱，〉14岁与≤14岁组间呈现不同的临床和免疫学异常特征，HSP器官累及与异常免疫学指标存在相关性。Objective To explore age-based clinical and immune parameters in Henoch-Sch6nlein purpura （HSP） to determine clinically useful markers reflecting disease characteristic. Methods A cohort of 502 patients with HSP were enrolled into this retrospective study to evaluate their clinical and immune data. Results Majority HSP cases occurred at age ≤14 years and showed significant immune imbalances of ESR, CD3＋ cells, CD4＋ cells, CD3 CD16＋CD56＋ cells, CD4＋/CD8＋ cells, IgG, IgA, IgM, IgE, complements C3/C4 and ASO in the acute phase. Compared to patients aged 〉14 years, symptoms of joint were more frequent at disease onset in patients aged ≤14 years （20.8% vs 7.6%, χ2=13.547, P〈0.001）, and involvement of digestive tract and joint were also more frequent （57.4% vs 33.8%, χ2=24.106, P〈0.001; 55.9% vs 32.5%, χ2=23.768, P〈0.001, respectively）, but not for involvement of kidney （21.4% vs 51.3%, χ2=42.440, P〈0.001）. The patients aged ≤14 years had distinct immune state, reductions of CD3＋ cells, CD4 cells and IgG were more frequent than patients aged 〉14 years, also increase of ASO （33.1% vs 20.0%, χ2=6.656, P=0.010）, but not increase of IgA （2.6% vs 39.4%, Z2=15.582, P〈0.001 ）. In addition, reduction of IgG and increase of IgE were positively associated with digestive tract involvement （P〈0.001,P=0.001, respectively）, reduction of CD3＋CD4＋ cells and normal IgM were positively associated with joint involvement （P=-0.004, P=0.003, respectively）, increase of CD3＋CD8＋ cells and normal CD3＋ cells were positively associated with kidney involvement （P=0.032, P=0.014, respectively）. Conclusion HSP showed significant immune imbalance in the acute phase, patients between aged ≤14 and 〉14 years had distinct clinical and immune characteristic, and abnormal immune parameters were significantly associated with organ involvements.

关 键 词：紫癜 过敏性 淋巴细胞亚群 免疫球蛋白类 补体C3 补体C4 

分 类 号：R554.6[医药卫生—血液循环系统疾病]