Effect of polymerization tendency on haematological, rheological and clinical parameters in sickle cell anaemia.
The polymerization tendency of sickle haemoglobin was estimated as a function of oxygen saturation in 30 patients with homozygous sickle cell anaemia. The deformability of their erythrocytes was also measured, by initial-flow-rate filtration at 37 degrees C through pores of 5 microns diameter, and clinical severity was assessed using a visual analogue scale. By means of partial correlation analysis, it was found that correlations between haematological, rheological, and clinical parameters in sickle cell anaemia could be explained on the basis of an association of each variable with polymerization tendency. Patients with the greatest tendency to form polymer had the least deformable erythrocytes and perceived their disease to be more severe as judged by the visual analogue scale. Polymer formation also appeared to be a determinant of the number of dense cells which, in turn, determine haemolytic rate and erythrocyte deformability.
Keidan
A J
AJ
Department of Haematology, Medical School, University of Birmingham, U.K.
Sowter
M C
MC
Johnson
C S
CS
Noguchi
C T
CT
Girling
A J
AJ
Stevens
S M
SM
Stuart
J
J
D000755:Anemia, Sickle Cell / Q000097:blood / Q000378:metabolism*; D004907:Erythrocyte Deformability; D005319:Fetal Hemoglobin / Q000032:analysis; D006441:Hemoglobin A / Q000032:analysis; D006451:Hemoglobin, Sickle / Q000378:metabolism*; D006801:Humans; D010100:Oxygen / Q000378:metabolism; D011108:Polymers; D012720:Severity of Illness Index
D006451:Hemoglobin, Sickle; D011108:Polymers; D006441:Hemoglobin A; D005319:Fetal Hemoglobin; D010100:Oxygen