Mean Corpuscular Hemoglobin Concentration in Hemoglobin CC, SC, and AC

Yan Zheng, Diana Castro, David Gay, Donghong Cai


Point mutation in the hemoglobin beta chain generates many deviants from the normal format hemoglobin A, which contains 2 alpha chains and 2 beta chains. Among the numerous deviants, Hemoglobin C (HbC) and Hemoglobin S (HbS, sickle cell hemoglobin) are 2 of the most common variants with clinical significance. Mean corpuscular hemoglobin concentration (MCHC) is of a measurement of the concentration of hemoglobin in a given volume of packed red blood cells. It is one of the red cell indices measured daily in every blood specimen, which provides clues to the clinicians about the quality of the red cells and the associated diseases. Previous studies have suggested that patients with HbCC and HbSC, and up to 45% patients with HbAC present with high MCHC. However, in our practice we found that the MCHC values in these patients showed a broad spectrum. To clarify this discrepancy, 388 peripheral blood specimens with HbCC (homozygous C), HbSC (compound heterozygous S and C), or HbAC (heterozygous C) were pulled out from hospital’s medical records. We found about 50% of the HbCC patients and 20% of HbSC patients had elevated MCHC, and in HbAC patients, elevation of MCHC was rare. Therefore, our study sheds new light on the interpretation of MCHC in daily practice.


MCHC, hemoglobinopathy, HbC, HbS, HbA

Full Text:



Charache S, Conley CL, Waugh DF, et al. Pathogenesis of hemolytic anemia in homozygous hemoglobin C disease. J Clin Invest. 1967;46:1795-1811.

Canterino JE, Galkin O, Vekilov PG, et al. Phase separation and crystallization of hemoglobin C in transgenic mouse and human erythrocytes. Biophys J. 2008;95:4025-4033.

Hirsch RE, Samuel RE, Fataliev NA, et al. Differential pathways in oxy and deoxy HbC aggregation/crystallization. Proteins. 2001;42:99-107.

Tokumasu F, Nardone GA, Ostera GR, et al. Altered membrane structure and surface potential in homozygous hemoglobin C erythrocytes. PLoS One. 2009;4(6):e5828.

Romero JR, Suzuka SM, Nagel RL, et al. Expression of HbC and HbS, but not HbA, results in activation of K-Cl cotransport activity in transgenic mouse red cells. Blood. 2004;103:2384-2390.

Ballas SK, Larner J, Smith ED, et al. The xerocytosis of Hb SC disease. Blood. 1987;69:124-130.

Ballas SK, Kocher W. Erythrocytes in Hb SC disease are microcytic and hyperchromic. Am J Hematol. 1988;28:37-39.

Nagel RL, Fabry ME, Steinberg MH. The paradox of hemoglobin SC disease. Blood Rev. 2003;17:167-178.

Cohen ML, Rifkind D. The pediatric abacus : Review of clinical formulas and how to use them. Edited by New York, NY, Parthenon Pub. Group, 2002, 110.

Hinchliffe RF, Ellis SP, Lilleyman JS. Discriminant function using red cell indices to distinguish between HbC and HbE traits. Clin Lab Haematol. 1995;17:31-33.

Hinchliffe RF, Norcliffe D, Farrar LM, et al. Mean cell haemoglobin concentration in subjects with haemoglobin C, D, E and S traits. Clin Lab Haematol. 1996;18:245-248.

Fabry ME, Kaul DK, Raventos-Suarez C, et al. SC erythrocytes have an abnormally high intracellular hemoglobin concentration. Pathophysiological consequences. J Clin Invest. 1982;70:1315-1319.

Hirsch RE, Rybicki AC, Fataliev NA, et al. A potential determinant of enhanced crystallization of Hbc: spectroscopic and functional evidence of an alteration in the central cavity of oxyHbC. Br J Haematol. 1997;98:583-588.

Vekilov PG, Feeling-Taylor AR, Petsev DN, et al. Intermolecular interactions, nucleation, and thermodynamics of crystallization of hemoglobin C. Biophys J. 2002;83:1147-1156.

Feeling-Taylor AR, Yau ST, Petsev DN, et al. Crystallization mechanisms of hemoglobin C in the R state. Biophys J. 2004;87:2621-2629.

Canessa M, Spalvins A, Nagel RL. Volume-dependent and NEM-stimulated K+,Cl- transport is elevated in oxygenated SS, SC and CC human red cells. FEBS Lett. 1986;200:197-202.

Charache S, Conley CL. Hereditary persistence of fetal hemoglobin. Ann N Y Acad Sci. 1969;165:37-41.

Lichtman MA, Kaushansky K, Kipps TJ, et al. Williams manual of hematology. Edited by New York, McGraw-Hill, 2011, 120-128.

Rodak BF. Hematology: clinical principles and applications. Edited by Philadelphia, W.B. Saunders, 2002, xvii, 835 p.

Rodak BF, Fritsma GA, Doig K. Hematology: clinical principles and applications. Edited by St. Louis, Saunders/Elsevier, 2007, xiii, 816 p.

Steinberg MH, Coleman MB, Adams JG, et al. The effects of alpha-thalassaemia in HbSC disease. Br J Haematol. 1983;55:487-492.

Webster P, Castro O. Red cell distribution width in sickle cell disease. Ann Clin Lab Sci. 1986;16:274-277.

Bourner G, Dhaliwal J, Sumner J. Performance evaluation of the latest fully automated hematology analyzers in a large, commercial laboratory setting: a 4-way, side-by-side study. Lab Hematol. 2005;11:285-297.


  • There are currently no refbacks.