The nonexpression of CD36 on reticulocytes and mature red blood cells does not modify the clinical course of patients with sickle cell anemia.
It is thought that an increase in the adhesion of circulating reticulocytes to the vascular endothelium may initiate the vascular occlusion underlying the painful crises and organ failures typical of sickle cell disease (SCD). At least 2 receptors, usually present on reticulocytes, seem to be involved in this adhesion process: glycoprotein CD36 (glycoprotein IV) and integrin alpha(4)beta(1) (very late activation antigen--4). Recently, a high frequency of the platelet CD36--deficient phenotype was reported in black Africans. The frequency of this deficiency was similar in subjects with and without SCD. The role of CD36 in vaso-occlusion was then investigated by comparing the clinical course in 2 groups of black Africans homozygous for hemoglobin S, with and without CD36 deficiency, but similar in age, sex, geographical origin, number of alpha-globin genes, and beta-globin gene haplotype. Flow cytometry showed that CD36 was absent from the circulating red blood cells and reticulocytes of platelet CD36--deficient individuals but present on those from patients with normal platelet CD36 expression, and that alpha(4)beta(1) integrin levels were similar on the reticulocytes of the 2 groups. Neither clinical severity, as evaluated by the frequency and characteristics of vaso-occlusive events, nor biological data differed significantly in the 2 groups of patients. Finally, although CD36 has been suggested to play a critical role in the pathogenesis of vaso-occlusion, this study, despite including only a small number of patients, supports the idea that the modulation of expression of a single type of adhesion molecule is insufficient to counteract the pathological process leading to vaso-occlusion in SCD patients. (Blood. 2001;98:966-971)
Lee
K
K
EFS-Ile de France, Laboratoire d'immunologie leuco-plaquettaire, Hôpital Henri Mondor, AP-HP, Créteil, France.
Gane
P
P
Roudot-Thoraval
F
F
Godeau
B
B
Bachir
D
D
Bernaudin
F
F
Cartron
J P
JP
Galactéros
F
F
Bierling
P
P
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000175:diagnosis / Q000628:therapy; D018955:CD36 Antigens / Q000096:biosynthesis*; D002648:Child; D002675:Child, Preschool; D004912:Erythrocytes / Q000737:chemistry*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D016555:Matched-Pair Analysis; D011379:Prognosis; D011446:Prospective Studies; D012156:Reticulocytes / Q000378:metabolism*; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric
D018955:CD36 Antigens