Mean Corpuscular Hemoglobin Concentration as a Discriminating Tool between Hereditary Spherocytosis and Intravascular Hemolysis

Purpose : The purposes of this study were to elucidate the diagnostic significance of mean corpuscular hemoglobin concentration(MCHC) for various kinds of hemolytic anemia, the mechanism of elevated MCHC in hereditary spherocytosis(HS). Methods : The subjects consisted of 39 cases of HS(group 1), 31 cases of intravascular hemolysis (IH) such as DIC, snake bite or hemolytic uremic syndrome(group 3), and sex and age matched controls(group 2, 4) for group 1, 3, respectively. The preand postoperative MCHC values were compared in 32 splenectomised HS patients. The age of subjects ranged from 1 to 18 years old. MCHC values were obtained with a H2 Technicon automated counter. Results : The MCHC was 34.5±2.3 g/dL, 33.0±1.1 g/dL, 32.4±2.0 g/dL and 32.7±1.0 g/dL in group 1, 2, 3, and 4, respectively. The MCHC of group 1 was significantly higher than those of group 2, 3 and 4(P<0.05). ROC curve showed that HS could be discriminated from IH or a normal control when MCHC was above 35 g/dL with a sensitivity of 0.51 and a specificity of 0.96. There was a significant difference between preand postoperative MCHC values(34.1±2.2 g/dL, 32.6±2.7 g/dL, respectively) in splenectomised HS patients(P<0.05). Conclusion : The MCHC obtained with laser scattering cytometry was elevated in cases of HS, and was a good diagnostic tool for discriminating HS from either IH or a normal control in children. The mechanism of elevated MCHC in HS seems to be the result of splenic processing. (J Korean Pediatr Soc 2001;44:664-668)