Partial complement deficiencies in idiopathic thrombocytopenia of childhood.
We have examined the classical complement activation pathway in 36 children with idiopathic thrombocytopenia (ITP) in a retrospective study. An increased prevalence of congenital, partial complement deficiencies is found in ITP. Homozygous C4A deficiency was found in 5 patients (p < 0.05) and heterozygous C2 deficiency in 2 other patients (p = 0.05). We suggest that some cases of childhood ITP belong to the immune complex mediated diseases, such as systemic lupus erythematosus and that abnormal immune complex formation and clearance may lead to ITP.
Nielsen
H E
HE
Department of Paediatrics, Rigshospitalet, Copenhagen, Denmark.
Truedsson
L
L
Donnér
M
M
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003175:Complement C2 / Q000172:deficiency*; D015934:Complement C4a / Q000172:deficiency*; D003171:Complement Pathway, Classical; D005787:Gene Frequency; D030342:Genetic Diseases, Inborn / Q000453:epidemiology* / Q000235:genetics; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D007223:Infant; D015995:Prevalence; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood / Q000145:classification / Q000150:complications*; D012008:Recurrence; D012189:Retrospective Studies
D003175:Complement C2; D015934:Complement C4a