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

Journal of the Korean Pediatric Society 2001;44(6):664-668.
Published online June 15, 2001.
Mean Corpuscular Hemoglobin Concentration as a Discriminating Tool between Hereditary Spherocytosis and Intravascular Hemolysis
Hae Young Park1, Hwang Min Kim1, Kyoung Ah Lee2, Dong Il Won2, Sei Myeng Park3, Seok Won Park1, Baek Keun Lim1, Kil Young Kim3
1Department of Pediatrics, Wonju College of Medicine, Yonsei University, Wonju, Korea
2Department of Clinical Pathology, Wonju College of Medicine, Yonsei University, Wonju, Korea
3Department of Pediatrics, College of Medicine, Yonsei University, Seoul, Korea
평균 적혈구 혈색소 농도를 이용한 유전성 구상적혈구증과 혈관내 용혈의 감별 진단
박혜영1, 김황민1, 이경아2, 원동일2, 박세명3, 박석원1, 임백근1, 김길영3
1연세대학교 원주의과대학 소아과학교실
2연세대학교 원주의과대학 임상병리학교실
3연세대학교 의과대학 소아과학교실
Abstract
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 pre- and 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¡¾2.3 g/dL, 33.0¡¾1.1 g/dL, 32.4¡¾2.0 g/dL and 32.7¡¾1.0 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 pre- and postoperative MCHC values(34.1¡¾2.2 g/dL, 32.6¡¾2.7 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.
Key Words: Mean coupscular hemoglobin concentration, Hereditary spherocytosis


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