title: Red blood cell indexes in patients in patients with hereditary spherocytosis and beta-thalassemia combination.
abstract: The spontaneous occurrence of hereditary spherocytosis (HS) and beta-thalassemia in the same patient is a rare event. The mean corpuscular hemoglobin concentration is elevated above the reference range in half to two-thirds of patients with HS, but there are no data for the HS/beta-thal combinations for the red blood cell indexes. This study reassessed these values in these particular patients. Hemoglobin, hematocrit, mean corpuscular volume (MCV), mean corpuscular hemoglobin concentration (MCHC), red cell distribution (RDW), and reticulocyte count were documented from 43 HS patients, 13 of which were from 10 families with the combination of beta-thal and HS; 28 controls were also included. Patients with HS/beta-thal have a significantly lower MCV, mean corpuscular hemoglobin, and MCHC and a significantly higher RDW than normal control subjects; 95% of beta-thalassemia carriers are free of clinical symptoms. When red blood cell indexes reveal a possibility of a beta-thalassemia carrier state with the symptoms of hemolytic anemia, HS should be considered.
authors.affiliation: Paediatric Molecular Genetics Department, Ankara University, Ankara, Turkey. nejatakar@hotmail.com
authors.forename: Nejat
authors.initials: N
authors.lastname: Akar
authors.forename: Hafize
authors.initials: H
authors.lastname: Gökçe
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D004252:DNA Mutational Analysis; D004909:Erythrocyte Indices / Q000502:physiology*; D005192:Family Health; D006403:Hematologic Tests; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D009154:Mutation; D013103:Spherocytosis, Hereditary / Q000097:blood / Q000150:complications* / Q000235:genetics; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications* / Q000235:genetics
chemical_list: D006454:Hemoglobins