Erythrocyte deformability and aggregation in homozygous sickle cell disease

Fecha de publicación: 01/01/2014

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

Investigador Principal

Javier De La Rubia Comos
Hemostasia, Trombosis, Arteriosclerosis y Biología vascular

Investigador Principal

Pilar Medina Badenes

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.

Datos de la publicación

ISSN/ISSNe:: 1386-0291, 1875-8622
Tipo:: Article
Páginas:: 497-505
DOI:: 10.3233/CH-131717
Factor de Impacto:: 0,810 SCImago ℠
Cuartil:: Q2 SCImago ℠

Documentos

No hay documentos

Métricas

Filiaciones

Vayá A:: Haemorheology and Haemostasis Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain
Collado S:: Haemorheology and Haemostasis Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain
Dasí MA:: Pediatry Service, La Fe University Hospital, Valencia, Spain
Pérez ML:: Haematology Service, La Fe University Hospital, Valencia, Spain
Hernandez JL:: Haemorheology and Haemostasis Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain
Barragán E:: Molecular Biology Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain

Keywords

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

Campos de estudio

Proyectos asociados

AVANCES EN EL ESTUDIO DE LA REACTIVIDAD PLAQUETARIA Y SU INTERACCION CON LOS ERITROCITOS: BASES MOLECUALRES Y APLICABILIDAD CLINICO-FARMACOLOGICA

Investigador Principal: JUANA VALLES GINER

PI03/0270 . INSTITUTO DE SALUD CARLOS III . 2004

RED DE INVESTIGACION COOPERATIVA DE CANCER

RD06/0020/0031 . INSTITUTO DE SALUD CARLOS III; FUNDACIÓN PARA LA INVESTIGACIÓN DEL HOSPITAL UNIVERSITARIO LA FE DE LA COMUNIDAD VALENCIANA . 2006

RED INVESTIGACION (RECAVA)

RD06/0014/0004 . INSTITUTO DE SALUD CARLOS III; FUNDACION PARA LA INV BIOMEDICA- HOSPITAL GREGORIO MARAÑON; FUNDACIÓN PARA LA INVESTIGACIÓN DEL HOSPITAL UNIVERSITARIO LA FE DE LA COMUNIDAD VALENCIANA . 2006