Erythrocyte deformability and aggregation in homozygous sickle cell disease

Autores de IIS La Fe

• Amparo Vaya Montañana

  Autor

• María De Los Ángeles Dasi Carpio

  Autor

• María Luz Perez Sirvent

  Autor

• Eva Barragán González

  Autor

Participantes ajenos a IIS La Fe

• Collado S
• Hernandez JL

Grupos

• Hematología y Hemoterapia

• Hemostasia, Trombosis, Arteriosclerosis y Biología vascular

Abstract

Rheological properties of homozygous sickle cell anaemia (SCA) showmarked heterogeneity, which may be explained in part by the concomitance of alpha genotypes or beta haplotypes, along with hydroxurea (HU) treatment. To further clarify this issue, in 11 homozygous patients with SCA in the steady state and in 16 healthy controls, we analysed erythrocyte deformability (ED) in a Rheodyn SSD by means of the Elongation Index (EI) at 12, 30 and 60 Pa, and erythrocyte aggregation at stasis (EA0) and at 3 sec(-1) (EA1) in a Myrenne aggregometer along with fibrinogen, biochemical and haematological parameters. When compared with controls, homozygous (SS) patients showed a lower EI at all the shear stresses tested (p < 0.01) and higher EA0 (p < 0.014), but not higher EA1 (p = 0.076). Fibrinogen did not show statistical differences (p = 0.642). In the Spearman's correlation IE60 correlated inversely with Hb S (p < 0.05) and directly with MCV, MCH and Hb F levels (p < 0.01). EA0 correlated inversely with MCV, MCH, Hb F (p < 0.01) and directly with Hb S (p < 0.05). HU treatment improved EI and EA0, but not EA1. This paradoxical behaviour of HU on erythrocyte aggregation merits further research to be clarified.

Keywords

• Sickle cell disease; erythrocyte deformability; erythrocyte aggregation; hydroxyurea treatment