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免疫相关性血细胞减少症41例临床分析
引用本文:陆沭华,张彦明,孙雨梅,何广胜,崔红霞,赵广圣,张兴霞. 免疫相关性血细胞减少症41例临床分析[J]. 临床血液学杂志, 2010, 0(2)
作者姓名:陆沭华  张彦明  孙雨梅  何广胜  崔红霞  赵广圣  张兴霞
作者单位:徐州医学院附属淮安医院江苏淮安市第二人民医院血液科;苏州大学附属第一医院血液科;
摘    要:
目的:观察41例免疫相关性血细胞减少(immunorelated hemocytopenia,IRH)或全血细胞减少症(immunorelated pancytopenia,IRP)患者的临床特点,以提高对本病的认识。方法:分析41例IRH/IRP患者临床表现、血常规、骨髓象、骨髓单个核细胞抗人球蛋白(BMMNC-Coombs)试验、抗核抗体ANA和ENA抗体谱、T淋巴细胞亚群、染色体核型等以及对肾上腺糖皮质激素、环孢素A等免疫抑制剂的治疗反应。结果:本组病例临床主要表现为贫血、出血、感染;血常规呈三系、二系或一系减少,网织红细胞百分比不低;骨髓增生以活跃或明显活跃为主(82.9%),红系比例多增高;BMMNC-Coombs试验阳性率78.0%;除外了再生障碍性贫血(AA)、骨髓增生异常综合征(MDS)、阵发性睡眠性血红蛋白尿(PNH)等疾病;小部分病例(19.5%)并发系统性红斑狼疮(SLE)等自身免疫性疾病;对肾上腺糖皮质激素、环孢素A等治疗有良好反应。结论:IRH/IRP是由于T淋巴细胞调控异常致B淋巴细胞产生针对骨髓未成熟造血细胞的自身抗体而使血细胞减少,BMMNC-Coombs试验对IRH/IRP的...

关 键 词:血细胞减少  自身免疫  骨髓单个核细胞  抗人球蛋白试验  

Clinical analysis of 41 patients with immune related hematocytopenia
LU Shuhua ZHANG Yanming SUN Yumei HE GuangshengCUI Hongxia ZHAO Guangsheng ZHANG Xingxia. Clinical analysis of 41 patients with immune related hematocytopenia[J]. Journal of Clinical Hematology, 2010, 0(2)
Authors:LU Shuhua ZHANG Yanming SUN Yumei HE GuangshengCUI Hongxia ZHAO Guangsheng ZHANG Xingxia
Affiliation:1Department of Hematology/a>;Second People Hospital of Huaian City/a>;Huaian/a>;223002/a>;China/a>;2Department of Hematology/a>;First Affiliated Hospital of Suzhou University
Abstract:
Objective:To improve the recognition of immune related hematocytopenia (IRH) or immune related pancytopenia (IRP) by observing clinical features of 41 patients with IRH/IRP.Method:Blood routine,bone marrow morphology,bone marrow mononuclear cell (BMMNC) antiglobulin test (Coombs test),antinuclear antibody ANA and ENA,subsets of T lymphocyte,chromosome analysis et al were performed in 41 cases of IRH/IRP/. Clinical features and responses to glucocorticoid,cyclosporin A were analyzed. Result:The clinical mani...
Keywords:hematocytopenia  autoimmunity  bone marrow mononuclear cell  antiglobulin test  
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