Rheological red blood cell behaviour in minor α-thalassaemia carriers. 2011

Amparo Vayá, and Marta Suescun, and Jose Luis Hernández, and María Luz Pérez, and Sarai Palanca, and Begoña Laiz
Hemorheology and Haemostasis Unit, Service of Clinical Pathology, La Fe University Hospital, Valencia, Spain. vaya_amp@gva.es

Although several studies have been published regarding rheological behaviour of red blood cells in beta and delta-beta thalassaemia traits, little information about erythrocyte deformability in alpha-thalassaemia carriers is available. We aimed to determine erythrocyte deformability in heterozygous (silent, -α/αα) and homozygous (minor alpha-thalassaemia, -α/-α) carriers of the alpha-thalassaemia trait for the alpha 3.7 deletion, the most common in our geographical area. We evaluated erythrocyte deformability by means of the elongation index (EI) in a Rheodyn SSD at 12, 30 and 60 Pa, along with basic haematological cell count, erythrocyte indices, reticulocytes, plasma lipids and iron metabolism parameters in 36 (18 women, 18 men) alpha-thalassaemia carriers (17 heterozygous, 19 homozygous) and 36 healthy subjects (23 women, 13 men). The molecular diagnosis of the alpha 3.7 deletion was evaluated by a PCR-based method. Alpha-thalassaemia carriers presented higher red blood cell counts, RDW-CV (p < 0.001) and lower haemoglobin, MCV, MCH and MCHC (p < 0.001) than controls. EI was statistically lower at 12, 30 and 60 Pa in cases than in controls (p = 0.001, p = 0.002, p = 0.010, respectively). No differences in either elongation indices or haematimetric values were observed when comparing silent heterozygous and minor homozygous alpha-thalassaemia carriers (p > 0.05). Pearson's bivariate correlation showed that EI60 correlated positively with haemoglobin and MCV, MCH, MCHC (p < 0.01), but negatively with ferritin (p< 0.05) and RDW-CV (p< 0.01). In the multivariate regression analysis, MCV (p = 0.001) and haemoglobin (p < 0.001) predicted EI60, with this model accounting for around 43% of variation in EI60 (R2 = 0.427). Alpha-thalassaemia carriers phenotypically showed mild microcytosis and hypochromia, irrespectively of them being silent heterozygous or minor homozygous alpha-thalassaemia carriers, which is associated with decreased erythrocyte deformability.

UI MeSH Term Description Entries
D008297 Male Males
D004903 Erythrocyte Aggregation The formation of clumps of RED BLOOD CELLS under low or non-flow conditions, resulting from the attraction forces between the red blood cells. The cells adhere to each other in rouleaux aggregates. Slight mechanical force, such as occurs in the circulation, is enough to disperse these aggregates. Stronger or weaker than normal aggregation may result from a variety of effects in the ERYTHROCYTE MEMBRANE or in BLOOD PLASMA. The degree of aggregation is affected by ERYTHROCYTE DEFORMABILITY, erythrocyte membrane sialylation, masking of negative surface charge by plasma proteins, etc. BLOOD VISCOSITY and the ERYTHROCYTE SEDIMENTATION RATE are affected by the amount of erythrocyte aggregation and are parameters used to measure the aggregation. Erythrocyte Aggregation, Intravascular,Agglutination, Intravascular,Intravascular Agglutination,Intravascular Erythrocyte Aggregation,Rouleaux Formation, Erythrocyte,Agglutinations, Intravascular,Aggregation, Erythrocyte,Aggregation, Intravascular Erythrocyte,Aggregations, Erythrocyte,Aggregations, Intravascular Erythrocyte,Erythrocyte Aggregations,Erythrocyte Aggregations, Intravascular,Erythrocyte Rouleaux Formation,Erythrocyte Rouleaux Formations,Formation, Erythrocyte Rouleaux,Formations, Erythrocyte Rouleaux,Intravascular Agglutinations,Intravascular Erythrocyte Aggregations,Rouleaux Formations, Erythrocyte
D004907 Erythrocyte Deformability Ability of ERYTHROCYTES to change shape as they pass through narrow spaces, such as the microvasculature. Erythrocyte Filterability,Deformability, Erythrocyte,Filterability, Erythrocyte
D004909 Erythrocyte Indices ERYTHROCYTE size and HEMOGLOBIN content or concentration, usually derived from ERYTHROCYTE COUNT; BLOOD hemoglobin concentration; and HEMATOCRIT. The indices include the mean corpuscular volume (MCV), the mean corpuscular hemoglobin (MCH), and the mean corpuscular hemoglobin concentration (MCHC). Erythrocyte Hemoglobin, Mean Cell,Erythrocyte Size Determination,Erythrocyte Volume, Mean Cell,Hemoglobin, Erythrocyte, Mean Cell,Mean Corpuscular Volume,Red Cell Indices,Erythrocyte Diameter,Erythrocyte Index,Erythrocyte Indexes,Erythrocyte Thickness,Mean Cell Hemoglobin Concentration,Mean Cell Volume,Mean Corpuscular Hemoglobin,Mean Corpuscular Hemoglobulin Concentration,Red Cell Distribution Width,Red Cell Index,Red Cell Indexes,Cell Volumes, Mean,Corpuscular Volumes, Mean,Determination, Erythrocyte Size,Determinations, Erythrocyte Size,Diameter, Erythrocyte,Diameters, Erythrocyte,Erythrocyte Diameters,Erythrocyte Size Determinations,Hemoglobin, Mean Corpuscular,Hemoglobins, Mean Corpuscular,Index, Erythrocyte,Index, Red Cell,Indexes, Erythrocyte,Indexes, Red Cell,Indices, Erythrocyte,Indices, Red Cell,Mean Cell Volumes,Mean Corpuscular Hemoglobins,Mean Corpuscular Volumes,Size Determination, Erythrocyte,Size Determinations, Erythrocyte,Thickness, Erythrocyte,Volume, Mean Cell,Volume, Mean Corpuscular,Volumes, Mean Cell,Volumes, Mean Corpuscular
D004912 Erythrocytes Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN. Blood Cells, Red,Blood Corpuscles, Red,Red Blood Cells,Red Blood Corpuscles,Blood Cell, Red,Blood Corpuscle, Red,Erythrocyte,Red Blood Cell,Red Blood Corpuscle
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D017085 alpha-Thalassemia A disorder characterized by reduced synthesis of the alpha chains of hemoglobin. The severity of this condition can vary from mild anemia to death, depending on the number of genes deleted. Hemoglobin H Disease,Thalassemia-alpha,A-Thalassemia,Alpha Thalassemia,Disease, Hemoglobin H,Thalassemia alpha,Thalassemia, Alpha,alpha-Thalassemias
D018056 Hemorheology The deformation and flow behavior of BLOOD and its elements i.e., PLASMA; ERYTHROCYTES; WHITE BLOOD CELLS; and BLOOD PLATELETS. Hemorrheology

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