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Rapid flow cytometric test for the diagnosis of membrane cytoskeleton‐associated haemolytic anaemia
Author(s) -
King MayJean,
Behrens Judith,
Rogers Chris,
Flynn Clare,
Greenwood David,
Chambers Keith
Publication year - 2000
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2000.02416.x
Subject(s) - hereditary spherocytosis , spherocytosis , spectrin , microbiology and biotechnology , medicine , band 3 , polyacrylamide gel electrophoresis , flow cytometry , pathology , chemistry , biology , membrane protein , immunology , biochemistry , membrane , cytoskeleton , enzyme , splenectomy , spleen , cell
The flow cytometric test measures the fluorescence intensity of intact red cells labelled with the dye eosin‐5‐maleimide, which reacts covalently with Lys‐430 on the first extracellular loop of band 3 protein. In this study, red cells from patients with hereditary spherocytosis (HS), congenital dyserythropoietic anaemia type II, South‐east Asian ovalocytosis and cryohydrocytosis have produced a greater degree of reduction of mean channel fluorescence readings than those for other patient groups and normal controls. The predictive value of this test for membrane abnormality was compared with the results obtained from the sodium dodecyl sulphate polyacrylamide gel electrophoresis (SDS–PAGE) method, which is currently the reference laboratory test for the identification of membrane protein deficiencies in hereditary spherocytosis and for the detection of spectrin variants in hereditary elliptocytosis. The dye method is a reliable, speedy diagnostic test (2 h from sample collection to result) for HS with a sensitivity of 92·7% and a specificity of 99·1%. Thus, it will serve well as a first‐line screening test for the diagnosis of hereditary spherocytosis in routine haematology.