与异常免疫相关的全血细胞减少症

目的 报告一组与异常免疫相关的全血细胞减少症。方法 分析29例骨髓单个核细胞Coombs试验阳性的血细胞减少症患者的临床及实验室检查特征。结果 该组患者大部分表现为全血细胞减少；绝大部分患者骨髓增生良好，少部分增生减低，但红系比例不低或增高；常规溶血试验阴性；无造血原料缺乏证据；无异常克隆造血证据；骨髓单个核细胞Coombs试验阳性；对肾上腺皮质激素为主的试验性治疗反应良好。结论 该组疾病与异常免疫（特别是自身抗体）介导的造血细胞破坏或功能异常有关，暂称为免疫相关性全血细胞减少症（immunorelated pancytopenia,IRP)，将其从再生障碍性贫血（AA）、骨髓增生异常综合征（MDS）中区分出来，患者可得到及时、有效的治疗，并且可减少误诊的AA、MDS。

Immunorelated pancytopenia

Objective A report of a group of patients with pancytopenia which might be related to abnormal immunity. Methods The clinical and laboratory features of 29 patients with positive bone marrow mononuclear cell(BMMNC) Coombs tests were analyzed. Results Most of these cases were pancytopenia with normal or decreased bone marrow cellularities and increased normoblasts. They were all found to have negative results of conventional hemolysis tests and hematopoietic nutrient determination and had no evidence of malignant clonal hematopoiesis. However, all of them showed positive BMMNC Coombs test and good response to corticosteroids. Conclusion Immunorelated pancytopenia might be caused by abnormal immunity (especially autoantibody) mediated destruction or abnormal function of blood cells. It is beneficial to differentiate immunorelated pancytopenia from aplastic anemia or myelodysplastic syndromes.