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Diagnosis of hereditary spherocytosis with dual-angle differential light scattering
F Gilsanz1, M P Ricard, I Millan
1Hospital 12 de Octubre, Universidad Complutense, Madrid, Spain.
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.
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.