title: Diagnosis of hereditary spherocytosis with dual-angle differential light scattering.
abstract: Efficacy in the diagnosis of hereditary spherocytosis (HS) using a system of dual-angle differential light scattering of a monochromatic laser beam on sphere-shaped and fixed erythrocytes was evaluated. Fifty-one nonsplenectomized patients with HS, 6 with autoimmune hemolytic anemia, and 140 control subjects were studied. The percentage of erythrocytes with hemoglobin concentration more than 410 g/L (hyperhemoglobin) was significantly different in patients with HS and control subjects (P < 0.001). Hyperhemoglobin could be detected even in the mildest HS, in all patients with autoimmune hemolytic anemia, and in some control subjects, although none of the latter had more than 3%. This technique is extremely sensitive to the presence of spherocytes and the absence of erythrocytes with more than 410 g/L hemoglobin excludes HS. The presence of hyperhemoglobin is not specific for HS and increased amounts of hyperhemoglobin were found in patients with autoimmune hemolytic anemia and control subjects. To make a confident diagnosis of HS, the detection of hyperhemoglobin must be evaluated in the same clinical setting and with the same criteria as those applied to the osmotic fragility test.
authors.affiliation: Hospital 12 de Octubre, Universidad Complutense, Madrid, Spain.
authors.forename: F
authors.initials: F
authors.lastname: Gilsanz
authors.forename: M P
authors.initials: MP
authors.lastname: Ricard
authors.forename: I
authors.initials: I
authors.lastname: Millan
mesh_terms: D004912:Erythrocytes / Q000473:pathology; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007834:Lasers; D012542:Scattering, Radiation; D013103:Spherocytosis, Hereditary / Q000097:blood / Q000175:diagnosis*
chemical_list: D006454:Hemoglobins