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Reduced deformability of erythrocytes as feature of congenital dyserythropoietic anaemia type II (HEMPAS).

Abstract
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).
AuthorsP Izzo, A Spagnuolo, A Manicone, P Nazzaro, V M Lauta
JournalClinical hemorheology and microcirculation (Clin Hemorheol Microcirc) Vol. 21 Issue 3-4 Pg. 425-30 ( 1999) ISSN: 1386-0291 [Print] Netherlands
PMID10711781 (Publication Type: Journal Article)
Chemical References
  • Hemoglobins
Topics
  • Adolescent
  • Anemia, Dyserythropoietic, Congenital (blood)
  • Erythrocyte Deformability
  • Erythrocyte Indices
  • Erythrocytes (metabolism, pathology, ultrastructure)
  • Female
  • Hemoglobins (metabolism)
  • Humans
  • Lasers
  • Male
  • Middle Aged
  • Optical Rotation

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