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的诊断具有较高的灵敏性和特异性,多数病例对肾上腺糖皮质激素等免疫抑制剂反应良好。
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 manifestations of 41 cases included anaemia,hemorrhage,infection,pancytopenia or hematocytopenia,but no reduce of the percentage of reticulocyte. Hematopoietic cells in bone marrow proliferated vigorously and the percentage of erythroid cells increased in most cases. The positive percentage of BMMNC-Coombs test was 78.0%. All patients were excluded of hematological system disease including aplastic anemia,myelodysplastic syndrome and paroxysmal nocturnal hemoglobinuria et al. Among 41 patients,19.5% were complicated with autoimmune diseases such as systemic lupus erythematosus. Satisfactory therapeutic responses to glucocorticoid and cyclosporin A appeared in most cases.Conclusion：Hematocytopenia in IRH/ IRP patients was caused by antibody against bone marrow immature cells. BMMNC-Coombs test had high sensitivity and specificity to IRH/ IRP. Most patients presented effective responses to glucocorticoid and cyclosporin A.
出处
《临床血液学杂志》
CAS
2010年第2期92-94,共3页
Journal of Clinical Hematology
