title: Intracellular Ca(2+)-containing vesicles in sickle cell disorders.
abstract: The frequency of red blood cells harboring Ca(2+)-containing vesicles was determined in patients with various sickle cell disorders; vesicles were identified by microscopy after the cells were stained with the fluorescent probe chlortetracycline. Specimens from 49 patients were studied. The highest frequencies of vesicle-containing cells were observed in samples from adults with homozygous sickle cell (SS) disease and in patients with S beta zero and SS(-alpha/alpha alpha) thalassemias. The frequency of cells with vesicles was less elevated in patients with SS(-alpha/-alpha) thalassemia and in patients with SS disease (Saudi Arabia high hemoglobin F), whereas a normal low frequency of positive cells was seen in patients with sickle cell-hereditary persistence of fetal hemoglobin and in patients with sickle trait. Deoxygenation induced an increase in the number of vesicle-containing cells that was proportional to the frequency of such cells in the oxygenated population. The frequency of Ca(2+)-containing vesicles in sickle red cells is associated with the clinical, hematologic, or clinical and hematologic severity of the sickle cell disorder.
authors.affiliation: Hematology/Oncology Unit, Mount Sinai Hospital, Chicago, IL 60608.
authors.forename: M P
authors.initials: MP
authors.lastname: Westerman
authors.forename: E
authors.initials: E
authors.lastname: Puchulu
authors.forename: R A
authors.initials: RA
authors.lastname: Schlegel
authors.forename: M
authors.initials: M
authors.lastname: Salameh
authors.forename: P
authors.initials: P
authors.lastname: Williamson
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000378:metabolism / Q000473:pathology; D002118:Calcium / Q000032:analysis* / Q000378:metabolism; D002648:Child; D004912:Erythrocytes / Q000737:chemistry / Q000378:metabolism / Q000648:ultrastructure; D006453:Hemoglobinopathies / Q000097:blood / Q000473:pathology; D006720:Homozygote; D006801:Humans; D007425:Intracellular Membranes / Q000737:chemistry* / Q000378:metabolism / Q000648:ultrastructure; D008856:Microscopy, Fluorescence; D012720:Severity of Illness Index; D017085:alpha-Thalassemia / Q000097:blood / Q000378:metabolism / Q000473:pathology; D017086:beta-Thalassemia / Q000097:blood / Q000378:metabolism / Q000473:pathology
chemical_list: D002118:Calcium