PMID- 6877904 OWN - NLM STAT- MEDLINE DCOM- 19830920 LR - 20041117 IS - 0031-3998 (Print) IS - 0031-3998 (Linking) VI - 17 IP - 6 DP - 1983 Jun TI - Hemoglobin Cheverly: an unstable hemoglobin associated with chronic mild anemia. PG - 503-7 AB - 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 degrees 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 Phe-Ser at position 45 (CD4) in the beta 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, beta 42 (CD1) Phe-Ser, 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. FAU - Yeager, A M AU - Yeager AM FAU - Zinkham, W H AU - Zinkham WH FAU - Jue, D L AU - Jue DL FAU - Winslow, R M AU - Winslow RM FAU - Johnson, M H AU - Johnson MH FAU - McGuffey, J E AU - McGuffey JE FAU - Moo-Penn, W F AU - Moo-Penn WF LA - eng PT - Case Reports PT - Journal Article PL - United States TA - Pediatr Res JT - Pediatric research JID - 0100714 RN - 0 (Hemoglobins, Abnormal) RN - 84837-27-4 (hemoglobin Cheverly) SB - IM MH - Anemia/etiology/*genetics MH - Child MH - Chronic Disease MH - Female MH - Hemoglobins, Abnormal/analysis/*isolation & purification MH - Humans MH - Reticulocytes/pathology EDAT- 1983/06/01 00:00 MHDA- 1983/06/01 00:01 CRDT- 1983/06/01 00:00 PHST- 1983/06/01 00:00 [pubmed] PHST- 1983/06/01 00:01 [medline] PHST- 1983/06/01 00:00 [entrez] AID - 10.1203/00006450-198306000-00016 [doi] PST - ppublish SO - Pediatr Res. 1983 Jun;17(6):503-7. doi: 10.1203/00006450-198306000-00016.