论文部分内容阅读
目的观察免疫相关性血细胞减少(immunorelated hematocytopenia,IRH)和免疫相关性全血细胞减少(immunorelated pancytopenia,IRP)患者HLA-B27群体表达情况,分析HLA-B27检测分型对该类疾病诊疗的临床意义。方法①ELISA方法检测患者血清HLA-B27抗原、IL-12、IL-17和IFN-γ水平。②细胞免疫化学(immunochemistry,IC)染色观察骨髓中抗原递呈细胞(APC)HLA-DR表达情况。③原位免疫荧光(immunofluorescence,IF)染色,观察患者骨髓造血细胞表面抗人球蛋白IgG、FcγⅡR、甘露糖受体(MR)、IL-12和IL-17A表达情况。④根据HLA-B27和血沉等检测指标对IRH/IRP分型选择治疗方案。结果①106例IRH/IRP中45例(42.45%)血清HLA-B27显著升高(70.03±8.21)pmol/L,对照组仅2例(6.0%),P<0.01。②HLA-B27+组血清IL-17和IFN-γ血清质量浓度分别为(50.26±3.67)pg/ml和(42.95±3.64)pg/ml,显著高于HLA-B27-组(36.11±2.17)pg/ml和(28.875±5.81)pg/ml,P值均<0.05。③IRH/IRP患者骨髓中树突状细胞(DC)和巨噬细胞(Mφ)捕获和吞噬血细胞现象活跃,活化的DC与Mφ高表达HLA-DR抗原,HLA-B27+组骨髓中可见到淋巴细胞攻击造血细胞现象;粒细胞减少的患者骨髓中嗜酸性粒细胞攻击粒细胞现象多见。④IRH/IRP患者骨髓中部分造血细胞表面表达抗人IgG,DC和Mφ表达MR、IL-12和IL-17A,并介导对病变造血细胞的捕获和吞噬和炎性损伤。⑤在伴有IgG和ESR升高的患者骨髓中活化的Mφ高表达FcγRⅡ受体,发挥ADCC效应介导对病变造血细胞的损伤。结论 IRH/IRP是一类涉及细胞免疫和体液免疫多个环节,主要累及骨髓造血细胞且具有基因易感性的免疫相关性血细胞减少性疾病。部分患者发病与HLA-B27基因活化状态有一定关联,HLA-B27+伴体液免疫指标活化者病情更重。HLA-B27检测对疾病的评估、治疗和预后有着重要的临床意义。
Objective To investigate the expression of HLA-B27 in patients with immunorelated hematocytopenia (IRH) and immunorelated pancytopenia (IRP) and to analyze the clinical significance of HLA-B27 typing in diagnosis and treatment of these diseases. Methods ① ELISA method for detection of serum HLA-B27 antigen, IL-12, IL-17 and IFN-γ levels. ② Immunochemistry (IC) staining was used to observe the expression of HLA-DR in antigen presenting cells (APC) in bone marrow. ③ In situ immunofluorescence (IF) staining was used to observe the expression of anti-human globulin IgG, FcγⅡR, mannose receptor (MR), IL-12 and IL-17A on bone marrow hematopoietic cells. ④ According to HLA-B27 and ESR and other indicators for the selection of IRH / IRP treatment options. Results ① Serum HLA-B27 was significantly increased (70.03 ± 8.21) pmol / L in 45 cases (42.45%) in 106 cases of IRH / IRP and only 2 cases (6.0%) in control group, P <0.01. ② Serum levels of IL-17 and IFN-γ in HLA-B27 + group were (50.26 ± 3.67) pg / ml and (42.95 ± 3.64) pg / ml respectively, significantly higher than those in HLA-B27- ml and (28.875 ± 5.81) pg / ml respectively, all P <0.05. ③ The phenomenon of capture and phagocytosis of dendritic cells (DCs) and macrophages (Mφ) in bone marrow of patients with IRH / IRP was active. HLA-DR antigens were expressed in activated DCs and Mφs, and lymphocytes were found in HLA-B27 + Hematopoietic cells; neutropenia in patients with bone marrow eosinophil attack granulocytes more common. ④ Anti-human IgG, DC and Mφ expressed MR, IL-12 and IL-17A on the surface of hematopoietic cells in the bone marrow of IRH / IRP patients and mediated the capture, phagocytosis and inflammatory damage of pathological hematopoietic cells. ⑤ Mφ, activated in the bone marrow of patients with elevated IgG and ESR, express FcγRⅡreceptors and play an ADCC effect to mediate the damage to diseased hematopoietic cells. Conclusion IRH / IRP is a kind of immune-related cytopenia disease involving multiple stages of cellular immunity and humoral immunity, mainly involving bone marrow hematopoietic cells and having gene susceptibility. Some patients with HLA-B27 gene activation and incidence of a certain relationship, HLA-B27 with humoral immune indicators activated more serious. HLA-B27 detection of disease evaluation, treatment and prognosis have important clinical significance.