Hemoglobin cheverly: An unstable hemoglobin associated with chronic mild anemia

Andrew M. Yeager, William H. Zinkham, Danny L. Jue, Robert M. Winslow, Mary H. Johnson, James E. McGuffey, Winston F. Moo-Penn

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Abstract

The evaluation of a family with chronic mild anemia led to the identification of a new unstable hemoglobin (Hemoglobin Cheverly). Modest anemia and reticulocytosis, normal to slightly increased mean corpuscular volume (MCV), and normal mean corpuscular hemoglobin concentration (MCHC) were present in the affected family members. Electrophoresis of blood samples on cellulose acetate and on citrate agar revealed normal patterns. Globin chain analysis and isoelectric focusing data were also normal. After incubation for 3 h at 41 °C, Heinz bodies were detected in 95-100% of erythrocytes from affected individuals. Positive heat and isopropanol tests confirmed the initial observation of the Heinz body preparation and indicated that an unstable hemoglobin was present. Structural analysis showed an amino acid substitution of PheSer at position 45 (CD4) in the p chain. Hemoglobin Cheverly has a reduced affinity for oxygen and a reduced Bohr effect, properties that can be rationalized on the basis of the x-ray crystallographic structure of normal hemoglobin. Despite structural and functional similarities between Hb Cheverly and Hb Hammersmith, β42 (CD1) PheSer, the clinical manifestations of Hb Cheverly are mild in contrast to the severe disease observed with Hb Hammersmith. Reasons for the apparently silent clinical expression of Hb Cheverly are not known. We discuss the implications of unstable hemoglobins in the evaluation of chronic anemia in pediatric patients.

Original languageEnglish (US)
Pages (from-to)503-507
Number of pages5
JournalPediatric Research
Volume17
Issue number6
DOIs
StatePublished - Jun 1983

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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    Yeager, A. M., Zinkham, W. H., Jue, D. L., Winslow, R. M., Johnson, M. H., McGuffey, J. E., & Moo-Penn, W. F. (1983). Hemoglobin cheverly: An unstable hemoglobin associated with chronic mild anemia. Pediatric Research, 17(6), 503-507. https://doi.org/10.1203/00006450-198306000-00016