Eberle Silvia Eandi, Sciuccati Gabriela, Bonduel Mariana, Díaz Lilian, Staciuk Raquel, Torres Aurora Feliú
Servicio de Hematología-Oncología, Hospital de Pediatría Juan P. Garrahan, Buenos Aires, Argentina.
Medicina (B Aires). 2007;67(6 Pt 2):698-700.
Hereditary spherocytosis is a group of heterogenous disorders characterized by variability in its clinical manifestations, membrane protein defects and inheritance. We analysed the sensitivity and specificity of mean corpuscular hemoglobin concentration (MCHC) and red cell distribution width (RDW) in the diagnostic screening of hereditary spherocytosis. Ninety-four patients were compared to equal number of healthy, age-matched children. All indexes were derived from measurements obtained by aperture impedance (Coulter Counter Model JT). In patients with hereditary spherocytosis, MCHC (35.67+/-1.33 g/dl) and RDW (20.60+/-4.5%) were significantly higher than in normal control subjects (MCHC 33.48+/-0.68 g/dl, p: 0.000; RDW 13.22+/-0.9%, p: 0.000). By using a cutoff for the MCHC of 34.5 g/dl and for the RDW of 14.5%, both indexes showed a sensitivity of 81% and a specificity of 98.9%. The combination of the two test is an excellent predictor for the diagnosis of hereditary spherocytosis.
遗传性球形红细胞增多症是一组异质性疾病,其临床表现、膜蛋白缺陷和遗传方式具有变异性。我们分析了平均红细胞血红蛋白浓度(MCHC)和红细胞分布宽度(RDW)在遗传性球形红细胞增多症诊断筛查中的敏感性和特异性。将94例患者与同等数量年龄匹配的健康儿童进行比较。所有指标均来自通过孔径阻抗法(库尔特计数器JT型号)获得的测量值。在遗传性球形红细胞增多症患者中,MCHC(35.67±1.33 g/dl)和RDW(20.60±4.5%)显著高于正常对照受试者(MCHC 33.48±0.68 g/dl,p:0.000;RDW 13.22±0.9%,p:0.000)。采用MCHC为34.5 g/dl和RDW为14.5%的临界值时,两个指标的敏感性均为81%,特异性均为98.9%。两项检测联合是遗传性球形红细胞增多症诊断的优秀预测指标。