[Erythrocyte indexes in hereditary spherocytosis].
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.
Eberle
Silvia Eandi
SE
Servicio de Hematología-Oncología, Hospital de Pediatría Juan P. Garrahan, Buenos Aires, Argentina.
Sciuccati
Gabriela
G
Bonduel
Mariana
M
Díaz
Lilian
L
Staciuk
Raquel
R
Torres
Aurora Feliú
AF
D002675:Child, Preschool; D016001:Confidence Intervals; D004909:Erythrocyte Indices; D005260:Female; D006454:Hemoglobins / Q000032:analysis*; D006801:Humans; D008297:Male; D008403:Mass Screening / Q000379:methods* / Q000592:standards; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D013103:Spherocytosis, Hereditary / Q000097:blood* / Q000175:diagnosis
D006454:Hemoglobins