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目的:研究疑难全血细胞减少症的免疫学指标改变,探讨其病因构成及预后。并研究骨髓Coombs试验在疑难全血细胞减少症病因诊断中的意义。方法:对36例疑难全血细胞减少症患者的免疫指标,T细胞亚群,骨髓Coombs试验结果进行分析,根据免疫指标的变化,给予部分患者试验性用药,出院后随访一年。另以25例再生障碍性贫血患者为再障对照组,以22例健康成人为健康对照组,比较骨髓Coombs试验阳性率的差异,X2检验P<0.05有统计学意义。结果:36位患者,18例诊断明确:5例为再生障碍性贫血,6例为免疫相关性全血细胞减少症,3例为系统性红斑狼疮,1例骨髓纤维化,1例肝癌,2例与妊娠相关的全血细胞减少;18例诊断不明确:8例患者于试验性升血治疗后血象好转,2例好转不明显,8例失访。骨髓Coombs试验阳性率在研究组与对照组的比较:疑难全血细胞减少症组(16.7%)高于再障对照组(X2=4.621,P<0.05),也高于正常对照组(X2﹦4.09,P<0.05)差异有统计学意义。结论:1对疑难全血细胞减少症患者进行试验性激素治疗及长期随访,有利于疾病的最终诊断。2免疫指标的检查及动态监测对明确疑难全血细胞减少症的病因十分重要。3骨髓Coombs试验对明确疑难全血细胞减少症病因有重要意义。
Objective: To study the changes of immunological parameters in patients with difficult pancytopenia and to explore its etiology and prognosis. And to investigate the significance of bone marrow Coombs test in the etiological diagnosis of difficult pancytopenia. Methods: The immune parameters, T cell subsets and bone marrow Coombs test results were analyzed in 36 cases of patients with difficult pancytopenia. According to the changes of immune indexes, some patients were given experimental drugs and were followed up for one year after discharge. Another 25 cases of aplastic anemia patients with aplastic anemia control group, 22 healthy adults as a healthy control group, comparison of bone marrow Coombs test positive rate differences, X2 test P <0.05 was statistically significant. RESULTS: Of the 36 patients, 18 had a clear diagnosis: 5 with aplastic anemia, 6 with immune-related pancytopenia, 3 with systemic lupus erythematosus, 1 with myelofibrosis, 1 with liver cancer and 2 with Pregnancy-related pancytopenia; 18 cases of diagnosis is not clear: 8 patients in the experimental blood after heliophagocytosis improved, 2 cases did not improve significantly, 8 cases were lost. The positive rate of bone marrow Coombs test in the study group and the control group: Difficult pancytopenia group (16.7%) was higher than the aplastic anemia control group (X2 = 4.621, P <0.05), also higher than the normal control group (X2 = 4.09 , P <0.05), the difference was statistically significant. Conclusions: 1 The experimental hormone therapy and long-term follow-up of patients with difficult pancytopenia are conducive to the final diagnosis of the disease. 2 immune index examination and dynamic monitoring of the clear difficult pancytopenia etiology is very important. 3 bone marrow Coombs test for clear cause of pancytopenia is of great significance.