[Immunological study in sickle cell disease patients: importance of the complement system].
Ten Tunisian patients, with homozygote sickle cell disease and asplenia were studied to investigate and to determine possible immunological function defects. Obtained results directed us to an abnormality of the alternate complement pathway activation which is expressed by a decreased hémolytic activity, while the classic pathway is normal. Quantification of C3, C4, C5, C6, C7 and factor B by immunochemical assay were normal, whereas factor B functional activity was depressed to a mean level of about half of normal in eight patients, IgG was increased in one subject and IgA in two others. Numeration of Band T cells revealed slight decrease in proportion of CD3 and CD4 at one patient associated with an increase in B cells, but normal or increased absolute numbers of all cells population.
Sassi
Fayçal
F
Laboratoire d'immunologie, EPS Charles Nicolle de Tunis.
Bardi
Rafika
R
Neji
Tebib
T
Ayed
Khaled
K
Ben Dridi
Marie Françoise
MF
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000235:genetics / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D003182:Complement C5 / Q000032:analysis; D003183:Complement C6 / Q000032:analysis; D003184:Complement C7 / Q000032:analysis; D011415:Complement Factor B / Q000032:analysis; D003165:Complement System Proteins / Q000032:analysis / Q000276:immunology*; D019085:Fluorescent Antibody Technique, Direct; D006720:Homozygote; D006801:Humans; D005779:Immunodiffusion; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D013601:T-Lymphocytes / Q000276:immunology
D003176:Complement C3; D003181:Complement C4; D003182:Complement C5; D003183:Complement C6; D003184:Complement C7; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D003165:Complement System Proteins; D011415:Complement Factor B