Red cell deformability, splenic function and anaemia in thalassaemia.
Red cell deformability (RCD) was measured in 38 patients with alpha-thalassaemia and 48 patients with beta-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 beta-thalassaemic patients (all patients r = 0.81, P < 0.001; homozygous beta-thalassaemic patients r = 0.51, P = 0. 01). These data suggest that reduced RCD is a major determinant of anaemia in thalassaemia.
Dondorp
A M
AM
Department of Internal Medicine and Division of Infectious Diseases, Tropical Medicine and AIDS, Academic Medical Centre, Amsterdam, The Netherlands.
Chotivanich
K T
KT
Fucharoen
S
S
Silamut
K
K
Vreeken
J
J
Kager
P A
PA
White
N J
NJ
D000740:Anemia / Q000097:blood*; D004907:Erythrocyte Deformability / Q000502:physiology*; D006801:Humans; D011182:Postoperative Care; D013156:Splenectomy; D013158:Splenic Diseases / Q000097:blood*; D013314:Stress, Mechanical; D017085:alpha-Thalassemia / Q000097:blood*; D017086:beta-Thalassemia / Q000097:blood*