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[免疫相关性血细胞减少症患者骨髓造血细胞自身抗体的类别、数量及临床意义]

[Category, quantity and clinical significance of autoantibodies on bone marrow hematopoietic cells in patients with immunorelated cytopenia].

作者信息

Fu Rong, Shao Zong-hong, Liu Hong, He Hong, Jia Hai-rong, Sun Juan, Zhao Ming-feng, He Guang-sheng, Shi Jun, Bai Jie, Chu Yu-lin, Yang Tian-ying

机构信息

Institute of Hematology and Blood Diseases Hospital, CAMS and PUMC, Tianjin 300020, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2003 Apr;24(4):177-80.

PMID:12864946
Abstract

OBJECTIVES

To explore the category, quantity and clinical significance of autoantibodies on bone marrow hematopoietic cells in patients with immunorelated cytopenia and evaluate the sensitivity of direct antiglobulin reaction (Coombs test ) of bone marrow mononuclear cells (BMMNC).

METHODS

The category and the positive rate of autoantibodies on bone marrow hematopoietic stem cells, nucleated erythrocytes, granulocytes in 32 patients with uncertain immunorelated cytopenia were investigated by using BMMNC-Coombs test and double immunofluorescence flow cytometry.

RESULTS

The positive rate of autoantibodies on bone marrow hematopoietic cells tested by flow cytometry was 90.63% which was higher than that by BMMNC-Coombs test (50.0%) (p < 0.05). In 29 positive cases, IgG autoantibody accounted for 6.90%, IgM13.8%, IgG+IgA 3.4%, IgG+IgM 31.0%, and IgG+IgM+IgA 44.8%. Of the 29 Patients, 25 (86.2%) with IgG autoantibody, 26 (89.7%) with IgM and 14 (48.3%) with IgA. The patients with IgG autoantibody alone had the lowest hemoglobin levels, and those with IgM autoantibody might have intravascular hemolytic findings. The response time of patients with IgG and IgG+IgM was shorter than that of the other patients. 91.3% of the patients had autoantibodies on bone marrow hematopoietic stem cells and showed pancytopenia, and 50% of the patients had autoantibodies on nucleated erythrocytes and granulocytes. Eleven of 13 patients with negative BMMNC-Coombs tests had autoantibodies on bone marrow hematopoietic stem cells detected by FACS. There was no significant difference of the quantities of the three categories of autoantibodies of nucleated erythrocytes and stem cells. The quantities of IgA on granulocytes were lower than that of IgG and IgM. There was no significant difference between IgG and IgM on granulocytes. The quantity of IgA on hematopoietic stem cells was significantly higher than that on nucleated erythrocytes or granulocytes.

CONCLUSIONS

The sensitivity of double immunofluorescence flow cytometry assay was higher than that of BMMNC-Coombs test for detecting autoantibodies. In immunorelated cytopenia patients, the predominant autoantibody was IgM which could cause intravascular hemolysis, and the second one was IgG which could cause severe anemia. Most immunorelated cytopenia patients had autoantibodies on hematopoietic stem cells and showed pancytopenia. IgA was more easily seen on the hematopoietic stem cells.

摘要

目的

探讨免疫相关性血细胞减少症患者骨髓造血细胞自身抗体的种类、数量及临床意义,并评估骨髓单个核细胞(BMMNC)直接抗球蛋白试验(Coombs试验)的敏感性。

方法

采用BMMNC - Coombs试验及双免疫荧光流式细胞术,检测32例免疫相关性血细胞减少症诊断不明确患者骨髓造血干细胞、有核红细胞、粒细胞上自身抗体的种类及阳性率。

结果

流式细胞术检测骨髓造血细胞自身抗体阳性率为90.63%,高于BMMNC - Coombs试验的50.0%(P < 0.05)。29例阳性病例中,IgG自身抗体占6.90%,IgM占13.8%,IgG + IgA占3.4%,IgG + IgM占31.0%,IgG + IgM + IgA占44.8%。29例患者中,单独IgG自身抗体者血红蛋白水平最低,IgM自身抗体者可能有血管内溶血表现。IgG及IgG + IgM患者的反应时间短于其他患者。91.3%的患者骨髓造血干细胞上有自身抗体并表现为全血细胞减少,50%的患者有核红细胞及粒细胞上有自身抗体。13例BMMNC - Coombs试验阴性患者中,11例通过流式细胞术检测到骨髓造血干细胞上有自身抗体。有核红细胞及干细胞这三类自身抗体的数量无显著差异。粒细胞上IgA的数量低于IgG和IgM。粒细胞上IgG和IgM无显著差异。造血干细胞上IgA的数量显著高于有核红细胞或粒细胞上的数量。

结论

双免疫荧光流式细胞术检测自身抗体的敏感性高于BMMNC - Coombs试验。在免疫相关性血细胞减少症患者中,主要的自身抗体是IgM,可引起血管内溶血,其次是IgG,可导致严重贫血。多数免疫相关性血细胞减少症患者骨髓造血干细胞上有自身抗体并表现为全血细胞减少。IgA在造血干细胞上更容易出现。

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