Rheological behaviour of red blood cells in beta and deltabeta thalassemia trait. 2003

Amparo Vayá, and Jesús Iborra, and Cristina Falcó, and Isabel Moreno, and Pascual Bolufer, and Fernando Ferrando, and María Luz Pérez, and Justo Aznar
Department of Clinical Pathology, La Fe University Hospital, Valencia, Spain. vaya_amp@gva.es

In major and intermediate thalassemia a decrease in erythrocyte deformability and increased erythrocyte aggregability has been described, but few studies have dealt with the question of rheological red blood cell behaviour in minor beta and deltabeta thalassemia carriers, mostly in deltabeta, because it is a less common entity. To ascertain whether there are differences in red blood cell behaviour between minor thalassemia and controls and between both types of thalassemia trait beta and deltabeta, we determined erythrocyte deformability and aggregability in 30 beta and 30 deltabeta trait carriers diagnosed both with conventional methods and globin gene analysis, and in 40 age- and sex-matched controls. Erythrocyte deformability determined by means of the Rheodyn SSD showed a statistically significant lower Elongation Index (EI) at all the shear stresses tested in both thalassemic groups compared with controls (p<0.001). Minor beta thalassemia carriers showed lower EI than deltabeta carriers (p<0.001). Erythrocyte aggregability measured with the Myrenne aggregometer was significantly lower in both thalassemic groups than in controls (p<0.001), although no significant differences could be observed between both thalassemic groups. The rheological alterations found in thalassemia carriers are in part due to microcytosis, hypochromia and the morphological changes that characterize this kind of anaemia. The less altered deformability found in deltabeta carriers, is in agreement with the fact that it deals with a more benign trait.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
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
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
D005340 Fibrinogen Plasma glycoprotein clotted by thrombin, composed of a dimer of three non-identical pairs of polypeptide chains (alpha, beta, gamma) held together by disulfide bonds. Fibrinogen clotting is a sol-gel change involving complex molecular arrangements: whereas fibrinogen is cleaved by thrombin to form polypeptides A and B, the proteolytic action of other enzymes yields different fibrinogen degradation products. Coagulation Factor I,Factor I,Blood Coagulation Factor I,gamma-Fibrinogen,Factor I, Coagulation,gamma Fibrinogen
D006579 Heterozygote An individual having different alleles at one or more loci regarding a specific character. Carriers, Genetic,Genetic Carriers,Carrier, Genetic,Genetic Carrier,Heterozygotes
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

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