CAJ | 학술논문

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。
목적보고일조여이상면역상관적전혈세포감소증。방법분석29례골수단개핵세포Coombs시험양성적혈세포감소증환자적림상급실험실검사특정。결과해조환자대부분표현위전혈세포감소;절대부분환자골수증생량호,소부분증생감저,단홍계비례불저혹증고;상규용혈시험음성;무조혈원료결핍증거;무이상극륭조혈증거;골수단개핵세포Coombs시험양성;대신상선피질격소위주적시험성치료반응량호。결론해조질병여이상면역(특별시자신항체)개도적조혈세포파배혹공능이상유관,잠칭위면역상관성전혈세포감소증(immunorelated pancytopenia,IRP), 장기종재생장애성빈혈(AA )、골수증생이상종합정(MDS)중구분출래,환자가득도급시、유효적치료,병차가감소오진적 AA、MDS。
Objective　A report of a group of patients with pancytopenia which might be related to abno rmal immunity. Methods　The clinical and laboratory features of 29 p atients with positive bone marrow mononuclear cell(BMMNC) Coombs tests were anal yzed. Results　Most of these cases were pancytopenia with normal o r decreased bone marrow cellularities and increased normoblasts. They were all f ound to have negative results of conventional hemolysis tests and hematopoietic nutrient determination and had no evidence of malignant clonal hematopoiesis. Ho wever, all of them showed positive BMMNC-Coombs test and good response to corti costeroids. Conclusion　Immunorelated pancytopenia might be cause d by abnormal immunity (especially autoantibody) mediated destruction or abnorma l function of blood cells. It is beneficial to differentiate immunorelated pancy topenia from aplastic anemia or myelodysplast ic syndromes.