儿童不典型再生障碍性贫血与ITP临床对比分析

ATYPICAL APLASTIC ANEMIA AND IMMUNE THROMBOCYTOPENIC PURPURA IN CHILDREN: A COMPARATIVE ANALYSIS

目的比较不典型再生障碍性贫血(AA)及免疫性血小板减少性紫癜(ITP)病儿临床特点,以减少早期误诊。方法收集本院收治44例不典型AA和48例ITP病儿的临床资料,对初诊时的外周血常规、骨髓常规、淋巴细胞亚群及应用糖皮质激素和(或)免疫球蛋白治疗4周后的疗效进行回顾性分析。结果不典型AA组淋巴细胞百分比(LYM)、平均红细胞体积(MCV)、红细胞体积分布宽度(RDW)、平均血红蛋白含量(MCH)、血小板数(PLT)、血小板压积(PCT)、血小板体积分布宽度(PDW)与ITP组比较,差异有显著性(Z=-7.664～-2.459,P<0.05)。不典型AA组中31例行骨髓涂片检查,其中2例为增生降低性骨髓,余为增生活跃性骨髓,而ITP组中38例均为增生活跃性骨髓。不典型AA组淋巴细胞比例增高者多于ITP组(χ2=16.048,P<0.05)。不典型AA组骨髓巨核细胞数、CD3+T细胞比例、CD19+B细胞比例与ITP组比较,差异有显著性(Z=-7.383～-4.979,P<0.05)。应用糖皮质激素和(或)免疫球蛋白治疗4周后,ITP组的治疗效果较不典型AA组好(Z=-3.455,P<0.05)。结论不典型AA外周血淋巴细胞比例、MCV、MCH、RDW、PDW增高,骨髓淋巴细胞比例增高,巨核细胞数增高不明显甚至降低,淋巴细胞亚群CD3+T细胞比例增高,CD19+B细胞比例不增高甚至降低,应用糖皮质激素和(或)免疫球蛋白治疗效果不佳,与ITP鉴别有一定指导价值。

Objective To compare the clinical characteristics between atypical aplastic anemia （AA） and immune throm- boeytopenic purpura （ITP）, so as to reduce early diagnostic errors. Methods The clinical data of 44 sick children with AA and 48 with ITP were collected. Their peripheral blood routine, bone marrow characteristics, lymphocyte subsets on preliminary diag- nosis and four weeks after glucocorticoid and/or immune globulin therapy were reviewed retrospectively. Results Differences between AA and ITP were significant in terms of lymphocyte ratio （LYM）, mean corpuscular volume （MCV）, red cell distribution width （RDW）, mean cell hemoglobin （MCH）, platelet count （PLT）, thrombocytocrit （TCC） and platelet distribution width （PDW） （Z= 7.664---2.459,P〈0.05）. In atypical AA,31 cases underwent bone marrow smear test, of whom, two showed hypoplastic bone marrow picture, the rest showed actively proliferated bone marrow picture; in ITP,38 examined were all with ac- tively proliferated picture. In atypical AA group, the ratio of cases with elevated lymphocyte was higher than that in ITP group （x2=16.048 ,P〈0.05）. The differences of the number of megakaryocyte, CD3＋ T cell ratio and CD19＋ B cell ratio between atypi cal AA and ITP were significant （Z=-7.383----4.979,P〈0.05）. After four-week therapy with glucocorticoid hormone and/or immune globulin, ITP responded better than atypical AA （Z= - 3.455,P〈0.05）. Conclusion In atypical AA, peri pheral blood LYM, MCV, MCH, RDW, PDW and bone marrow lymphocyte ratio increased, the number of megakaryocyte increased not markedly and even decreased, the ratio of CD3＋ T cells increased, CD19＋ B cells did not increased or even decreased, which did not response well to the therapy with glucocorticoid hormone and/or immune globulin. A differentiation between atypical AA and ITP has certain instructional significance.