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Partial complement deficiencies in idiopathic thrombocytopenia of childhood
Author(s) -
Nielsen HE,
Truedsson L,
Donner M
Publication year - 1994
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1994.tb13132.x
Subject(s) - medicine , complement deficiency , immune thrombocytopenia , complement system , complement (music) , immunology , classical complement pathway , immune system , c4a , lupus erythematosus , alternative complement pathway , complement c1q , antibody , phenotype , biochemistry , chemistry , complementation , gene
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.