Reduced deformability of erythrocytes as feature of congenital dyserythropoietic anaemia type II (HEMPAS).
In this study we report results regarding erythrocytes deformability in congenital dyserythropoietic anemia type II (HEMPAS) by the use of LORCA (Laser-assisted Optical Rotational Cell Analyzer). The reduced erythrocytes deformability observed in seven case of CDA II is caused by changes in the structure of glycoproteins due to the incomplete glycosylation of erythrocytic and erythroblastic membrane. Erythrocytes deformability (EI) was shown to be inversely related with mean corpuscular volume (MCV) and mean haemoglobin concentration (MCH).
Izzo
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DIMO, Biomedical Science and Human Oncology, University of Bari, Italy.
Spagnuolo
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Manicone
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Nazzaro
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Lauta
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VM
D000293:Adolescent; D000742:Anemia, Dyserythropoietic, Congenital / Q000097:blood*; D004907:Erythrocyte Deformability; D004909:Erythrocyte Indices; D004912:Erythrocytes / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D007834:Lasers; D008297:Male; D008875:Middle Aged; D009904:Optical Rotation
D006454:Hemoglobins