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

Article type: Research Article

Authors: Izzo, P.^; | Spagnuolo, A. | Manicone, A. | Nazzaro, P. | Lauta, V.M.

Affiliations: DIMO, Biomedical Science and Human Oncology, University of Bari, Bari, Italy | Internal Medicine and Hypertension, University of Bari, Bari, Italy

Note: [] Corresponding author: P. Izzo, Biomedical Science and Human Oncology, University of Bari, Palazzo Ateneo – piazza Umberto I, 1, 70122 Bari, Italy.

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 deformbility (EI) was shown to be inversely related with mean corpuscular volume (MCV) and mean haemoglobin concentration (MCH).

Keywords: Dyserythropioietic anaemia, LORCA, erythrocytes deformability

Journal: Clinical Hemorheology and Microcirculation, vol. 21, no. 3-4, pp. 425-430, 1999