High-level serum B-cell activating factor and promoter polymorphisms in patients with idiopathic thrombocytopenic purpura.
Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder in which platelets are opsonised by autoantibodies and destroyed by macrophages. Therefore, ITP represents a prototype of a B-cell-mediated autoimmune disorder. B-cell activating factor (BAFF) is a member of the tumour necrosis factor family and an important regulator of B-cell development. BAFF levels were determined in serum samples from 53 patients with ITP. Serum BAFF levels in patients with an active ITP were increased when compared with the healthy control group (median 1620 pg/ml vs. 977 pg/ml; P < 0.001). Moreover, immunosuppressive treatment was associated with strongly suppressed BAFF levels (median 629 pg/ml; P < 0.01). In addition, a polymorphic site was detected in the BAFF promoter region (-871) that appeared to occur more frequently in ITP patients than in healthy persons. This promoter variant was associated with very high BAFF levels in ITP patients. Our data suggest that BAFF is an important pathogenetic factor in the development of ITP.
Emmerich
Florian
F
Institute for Transfusion Medicine, Charité University Medicine Berlin, Berlin, Germany. florian.emmerich@charite.de
Bal
Gürkan
G
Barakat
Alaa
A
Milz
Julian
J
Mühle
Caroline
C
Martinez-Gamboa
Lorena
L
Dörner
Thomas
T
Salama
Abdulgabar
A
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D053264:B-Cell Activating Factor / Q000097:blood* / Q000235:genetics*; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D002908:Chronic Disease; D005260:Female; D020022:Genetic Predisposition to Disease; D016678:Genome; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D020641:Polymorphism, Single Nucleotide; D011239:Prednisolone / Q000627:therapeutic use; D011401:Promoter Regions, Genetic; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood / Q000188:drug therapy / Q000235:genetics*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D017422:Sequence Analysis, DNA; D018709:Statistics, Nonparametric
D053264:B-Cell Activating Factor; D015415:Biomarkers; D007166:Immunosuppressive Agents; D011239:Prednisolone