免疫相关性全血细胞减少症的实验研究

【作者】 付蓉；

【导师】 邵宗鸿；

【作者基本信息】 中国协和医科大学 ， 内科学， 2002， 博士

【摘要】 目的 (1)了解免疫相关性全血细胞减少症(IRP)患者骨髓造血细胞结合的自身抗体的类型、分布、数量及临床意义 (2)考察骨髓单个核细胞直接抗人球蛋白试验(BMMNC-Coombs)的敏感性。 方法 对临床疑诊32例IRP患者同时进行BMMNC-Coombs试验和流式细胞术(Facs)双标法检测骨髓造血干祖细胞、有核红细胞、粒细胞结合的自身抗体的种类及结合率。并观察其与临床特征间的联系。 结果 (1)Facs双标法检出的骨髓造血细胞自身抗体的阳性率为90.63％，BMMNC-Coombs试验检出的阳性率为50％，前者显著高于后者(x~2=12.65，P＜0.05)。 (2)Facs检测阳性的29例患者中IgG型占6.90％，IgM型占13.8％，IgG+IgA型占3.45％，IgG+IgM型占31.0％，IgG+IgM+IgA型占44.8％；29例中含IgG型25例，占86.2％，含IgM型26例，占89.7％，含IgA型14例，占48.3％； (3)IgG型组血红蛋白(Hb)减少得最严重，含IgM型组的患者可有血管内溶血的实验发现，IgM和IgM+IgG+IgA型组治疗的起效时间明显短于其它两组。 (4)在Facs检测阳性的29例患者中造血干祖细胞上有自身抗体者占91.3％，多表现为全血细胞减少；50％左右的患者红、粒系细胞上有自身抗体；13例BMMNC-Coombs试验阴性的患者中更多还原

【Abstract】 Objectives 1 ) To explore the type, distribution, quantity, and clinical significance of auto-antibodies on bone marrow hematopoietic cells in patients with immunorelated pancytopenia (IRP); 2) To evaluate the sensitivity of bone marrow mononuclear cell (BMMNC) Coombs tests. Methods The type and the positive rate of autoantibodies on bone marrow hematopoietic stem cells, nucleated erythrocytes, granulocytes of patients with suspected IRP were investigated with BMMNC-Coombs tests and flow cytometry double immunofluorescence assay.Results 1 ) The positive rate of auto-antibodies on bone marrow hematopoietic cells tested with flow cytometry was 90.63% which was significantly higher than that tested with BMMNC-Coombs test (50%). 2) In 29 cases with positive results, only IgG autoantibody positive cases accounted for 6.90%, only IgM accounted for13.8%, IgG+IgA accounted for 3.45%, IgG+IgM accounted for 31.0%, IgG+IgM+IgA accounted for 44.8%; 25 patients with IgG autoantibody accounted for 86.2%, 26 patients with IgM accounted for 89.7%, 14 patients with IgA accounted for 48.3%. 3) The patients only with IgG autoantibody had the most severe hemoglobin decrease, the patients with IgM autoantibody had the positive hemolytic findings, the response time of patients with IgM and IgG+IgM+IgA is shorter than that of the other patients. 4) 91.3% of the patients had auto-antibodies on bone marrow hematopoietic stem cells and presented with pancytopenia; 50% of the patients had auto-antibodies on nucleated erythrocytes and granulocytes, 11 of 13 pattients with negative BMMNC-Coombs tests had更多还原