Red cell deformability, splenic function and anaemia in thalassaemia

• Published in: British journal of haematology Vol. 105; no. 2; pp. 505 - 508
• Main Authors: Dondorp, A. M., Chotivanich, K. T., Fucharoen, S., Silamut, K., Vreeken, J., Kager, P. A., White, N. J.
• Format: Journal Article
• Language: English
• Published: Oxford, U.K. and Cambridge, USA Blackwell Science Ltd 01.05.1999; Blackwell; Blackwell Publishing Ltd
• Subjects: alpha-Thalassemia - blood; anaemia; Anemia - blood; Anemias. Hemoglobinopathies; beta-Thalassemia - blood; Biological and medical sciences; Diseases of red blood cells; Erythrocyte Deformability - physiology; Hematologic and hematopoietic diseases; Hematology; Humans; Medical sciences; Postoperative Care; red cell deformability; Splenectomy; Splenic Diseases - blood; splenic function; Stress, Mechanical; thalassaemia; Human; Spleen; Hemoglobinopathy; Hemolytic anemia; Deformability; Pathophysiology; Rheology; Red blood cell; Thalassemia; Hemopathy; Blood; Genetic disease
• ISSN: 0007-1048; 1365-2141
• DOI: 10.1111/j.1365-2141.1999.01367.x

Red cell deformability (RCD) was measured in 38 patients with α‐thalassaemia and 48 patients with β‐thalassaemia, of whom 13 had undergone splenectomy. All splenectomized patients, but none of those with intact spleens, had very rigid erythrocytes with an elongation index <0.45 at a high shear stress of 30 Pa suggesting a splenic recognition threshold for removal of rigid red cells. At this shear stress RCD correlated strongly with the degree of anaemia in both the splenectomized (r = 0.81, P < 0.001) and non‐splenectomized β‐thalassaemic patients (all patients r = 0.81, P < 0.001; homozygous β‐thalassaemic patients r = 0.51, P = 0.01). These data suggest that reduced RCD is a major determinant of anaemia in thalassaemia.