【作者】 贺远；

【Author】 He Yuan

【机构】 贵州省人民医院血液科；

【摘要】 目的总结17例免疫相关性全血细胞减少症(immunorelate pancytopenia,IRP)患者临床特点,了解本病临床诊断要点及治疗方法,减少误诊、漏诊。方法用常规的临床资料及实验室检查排除再生障碍性贫血(aplastic anemia,AA),骨髓增生异常综合征(myelodysplastic syndrome,MDS),阵发性睡眠性血红蛋白尿(paroxysmal nocturnal hemoglobinuria,PNH),营养不良性贫血,自身免疫性溶血性贫血,Evan’s综合征等常见血细胞减少性疾病。检测骨髓单个核细胞抗人球蛋白试验(BMMNC-Coomb’s)并用肾上腺皮质激素或大剂量静脉丙种球蛋白、环孢菌素治疗。将治疗有效者进行临床分析。结果该组全血细胞减少症不符合其他可能引起全血细胞减少的疾病,特点为全血细胞减少,网织红细胞和中性粒细胞百分数不低,骨髓多数增生不减低,红系比例正常或增高,常规溶血检查为阴性,没有异常克隆造血证据,对肾上腺皮质激素或大剂量丙种球蛋白治疗敏感,环孢菌素可巩固疗效。尽管BMMNC-Coomb’s试验阴性,它们仍符合IRP的诊断标准。结论该组疾病与异常免疫相关,网织红细胞和中性粒细胞百分数不低,对肾上腺皮质激素或大剂量丙种球蛋白治疗有效。但由于自身抗体存在于早期造血干细胞,数量少,BMMNC-Coomb’s试验阳性率不高,并且影响该试验的因素较多,故BMMNC-Coomb’s试验阴性者不能排除。需进一步改善检测方法,有条件者可用流式方法提高阳性检测率。更多还原

【Abstract】 Objective To make a summary on the clinical characters of 17 immunorelate pancytopenia(IRP)patients and understand the key of clinical diagnosis and therapies of this disease so as to avoid misdiagnosis and missed diagnosis.Methods Use the conventional clinical data and laboratory examination to deplete such common hematocytopenia diseases as aplastic anemia(AA), myelodysplastic syndrome(MDS),paroxysmal nocturnal hemoglobinuria(PNH), malnutritional anemia,autoimmune hemolytic anemia and Evan’s symptom complex and so on.Then make a BMMNC Coomb’s detection and treat the patients with adrenal cortex hormone(ACH)or large doses of gamma globulin and cyclosporine.After that a clinical analysis is made on the responders.Results Different from those of other pancytopenia patients,the symptoms of the tested group patients are featured with pancytopenia,high percentage of granulofilocyte and heterophil granulocyte, accrementition non-abaissement of most BM and normal or increased erythrocyte system. At the same time the routine haematolysis test is negative without abnormal clone haematogenesis and the patients in this test are sensitive to adrenal cortex hormone or flushing dose of gamma globulin.It is found that ciclosporin can strengthen the therapeutic effect.Though the BMMNC-Comb’s test is negative and these patients are still consistent with IRP diagnostic criteria.Conclusion This disease is related with abnormal immune;its percentage of granulofilocyte and heterophil granulocyte is not low and it responds to adrenal cortex hormone or flushing dose of gamma globulin.But due to the small amount of haemopoietic stem cells,the low rate of BMMNC-Coomb’s positive reaction and many other factors which effect the experiment,the negative of BMMNC-Coomb’s cannot be expelled.It is necessary to improve the test methods and flow cytometry can be used to improve the rate of positive reaction.更多还原